Добавил:

Sekretar kiopkiopkiop18@yandex.ru t.me/Prokururor I Вовсе не секретарь, но почту проверяю Опубликованный материал нарушает ваши авторские права? Сообщите нам.

Вуз:

Ростовский Государственный Медицинский Университет

Предмет:

Медицина общая

Файл:

Книга_4446_Библиотеки_им_академика_М_И_Перельмана

.pdf

Скачиваний:

Добавлен:

30.08.2026

Размер:

49 Мб

Скачать

☆

<<< < Предыдущая 1 2 3 4 5 6 7 8 9 10 1112 / 5012 13 14 15 16 17 18 19 20 21 22 23 24 25 > Следующая >>>

8 Otosclerosis

101

Fig. 8.13 Right ear otosclerosis with the persistent stape-

dial artery (PSA). The posterior half of the footplate is visible (asterisk) and could be exceptionally accessible for fenestration

2. Dizziness or vertigo: it can be due to labyrinthitis, which is self-limited and resolves within 3–6days [39]. Other factors can cause dizziness like the heating effect of laser, long prosthesis, and depressed footplate fragment [40]. Long-lasting dizziness requires exploration to rule out a perilymph stula.

3. Sensorineural hearing loss: less than 1% in experienced hands [41]. It can be caused by perilymph stula, granuloma formation, or very long prosthesis that penetrates deep into the vestibule (Fig.8.14).

4. Perilymph stula: primary perilymph stula occurs just after the fenestration and persists long after, while secondary perilymph stula appears after a period of time of successful sealing. The latter is usually due to the dislocation of the prosthesis. Perilymph stula indicates explorative tympanotomy.

5. Reparative granuloma: it is a reactive granulation tissue that occurs after the surgery. It can invade the vestibule leading to hearing loss, tinnitus, and dizziness that appear after 7–12days after the surgery. Most of the cases end in a profound permanent sensorineural hearing loss (Fig.8.15).

Fig. 8.14 Reformatted CT-Image along the prosthesis

axis: stapes prosthesis laterally in contact with the long process of incus and the tympanic membrane (empty arrow), medially the long piston of the stapes prosthesis penetrates deep into the vestibule (white arrow). Thickened footplate between the black arrows

Fig. 8.15 Reformatted CT along the prosthesis axis:

hazy condensations around the piston (white arrows) due to the proliferative inammatory tissue of the granuloma. Intravestibular tip of the prosthesis: black arrow

6. Facial palsy: an immediate post stapedectomy facial palsy is usually due to excessive local anesthesia inltration. Delayed palsy is usually due to reactivation of the VZ virus or Bell’s palsy.

7. Otitis media: Rare, but can cause sensorineural hearing loss. The patient should be admit-

102

Данная книга находится в списке для перевода на русский язык сайта https://meduniver.com/

ted to the hospital to receive antibiotics and steroid. Ear packs, if still in the canal, should be removed.

8.9.5.1 Failure inStapes Surgery

Causes of persistent conductive hearing loss

1. Malleus ankylosis

2. Round window otosclerosis

3. Third window Short prosthesis or

4. small diameter prosthesis

Prosthesis abutting fenestration margin

Causes of recurrent conductive hearing loss

Resorptive osteitis of the incus

2. Displacement of the prosthesis New bone formation in

3. the oval window

Obliteration of the round window by otosclerosis

The most common cause to perform revision stapes surgery is prosthesis displacement followed by incus erosion.

8.10 Conservative Treatment ofHearing Loss inOtosclerosis

1. Hearing aid.

2. BAHA or middle ear implant.

3. Medical treatment: Given in cases of active

otosclerosis. Signs and symptoms like the presence of Schwartz sign and onset or worsening of tinnitus, vertigo, or hearing loss can indicate active disease. The options for medical therapy include:

(a) Sodium Fluoride: change the active lesion

to an inactive lesion.

(b) Bisphosphonates: it induces apoptosis in

osteoclasts, reducing toxic enzymes’ production secondary to abnormal bone metabolism.

Take-Home Messages

• Otosclerosis typically presents with slowly progressive unilateral or bilateral deafness, tinnitus, and vertigo may also occur.

• The hearing loss encountered in these patients can be conductive, sensorineural, or mixed.

S. Mansour et al.

• In patients with typical clinical features and uncomplicated conductive hearing loss, audiometry tests are mandatory to establish the diagnosis.

• Imaging study is helpful to determine the disease stages and differential diagnosis, assess associated anatomic abnormalities, and evaluate postoperative prosthesis status.

References

1. Schuknecht HF, Barber W.Histologic variants in otosclerosis. Laryngoscope. 1985;95:1307–17.

2. Altmann F, Glasgold A, Macduff JP. The incidence of otosclerosis as related to race and sex. Ann Otol Rhinol Laryngol. 1967;76:377–92.

3. Cawthorne T. Otosclerosis. J Laryngol Otol. 1955;9:437–56.

4. Precechtel A.Determination of the effect of pregnancy on the activation of otosclerosis. Acta Otolaryngol. 1967;63:121–7.

5. Zehnder AF, Kristiansen AG, Adams JC, Kujawa SG, Merchant SN, McKenna MJ.Osteoprotegrin knockout mouse demonstrate abnormal remodeling of the otic capsule and progressive hearing loss. Laryngoscope. 2006;116:201.

6. Sørensen MS, Jørgensen MB, Bretlau P.Drift barriers in the postcartilaginous development of the mammalian otic capsule. Eur Arch Otorhinolaryngol. 1992;249:56–61.

7. Mansour S, Nicolas K, Ahmad HH.Round window otosclerosis: radiologic classication and clinical correlations. Otol Neurotol. 2011;32:384–92.

8. Moumoulidis I, Axon P, Baguley D, Reid E.A review on the genetics of otosclerosis. Clin Otolaryngol. 2007;32(4):239–47. Review.

9. Niedermeyer HP, Gantumur T, Neubert WJ, Arnold W. Measles virus and otosclerosis. Adv Otorhinolaryngol. 2007;65:86–92.

10. Yoo TJ. Etiopathogenesis of otosclerosis: a hypothesis. Ann Otol Rhinol Laryngol. 1984;93:28–33.

11. Cureoglu S, Schachern PA, Ferlito A, Rinaldo A, Tsuprun V, Paparella MM. Otosclerosis: etiopathogenesis and histopathology. Am J Otolaryngol. 2006;27(5):334–40.

12. Valvassori GE.Imaging of otosclerosis. Otolaryngol Clin N Am. 1993;26:359–71.

13. Hueb MM, Goycoolea MV, Paparella MM, Oliveira JA. Otosclerosis: the University of Minnesota temporal bone collection. Otolaryngol Head Neck Surg. 1991;105:396–405.

14. Schuknecht HF, Kirchner JC.Cochlear otosclerosis: fact or fantasy? Laryngoscope. 1974;84:766–82.

AL GRAWANY

8 Otosclerosis

103

15. van Loon MC, Merkus P, Smit CF, Smits C, Witte BI, Hensen EF. Stapedotomy in cochlear implant candidates with far advanced otosclerosis: a systematic review of the literature and meta-analysis. Otol Neurotol. 2014;35(10):1707–14.

16. Gristwood RE, Venables WN. Otosclerosis and chronic tinnitus. Ann Otol Rhinol Laryngol. 2003;112:398–403.

17. Sando I, Hemenway WG, Miller DR, Black FO. Vestibular pathology in otosclerosis temporal bone histopathological report. Laryngoscope. 1974;84:593–605.

18. Paparella MM, Chasin WD.Otosclerosis and vertigo. J Laryngol Otol. 1966;80:511–9.

19. Hannley MT. Audiologic characteristics of the patient with otosclerosis. Otolaryngol Clin N Am. 1993;26(3):373–87.

20. Bel J, Causse J, Michaux P, Cézard R, Canut Y, Tapon J.Mechanical explanation of the on-off effect (diphasic impedance change) in otospongiosis. Audiology. 1976;15(2):128–40.

21. Lagleyre S, Sorrentino T, Calmels MN, Shin YJ, Escudé B, Deguine O, Fraysse B.Reliability of highresolution CT scan in diagnosis of otosclerosis. Otol Neurotol. 2009;30(8):1152–9.

22. Révész P, Liktor B, Liktor B, Sziklai I, Gerlinger I, Karosi T.Comparative analysis of preoperative diagnostic values of HRCT and CBCT in patients with histologically diagnosed otosclerotic stapes footplates. Eur Arch Otorhinolaryngol. 2016;273(1):63–72.

23. Ariadna M, Cozma S, Murariu O, Radulescu L, Haba MSC, Vreme RM, Haba D, Iasi/ RO.ECR 2017 diagnostic value of CBCT in otosclerosis. Poster No 2283;

2017.

24. Fisch U. Stapedotomy versus stapedectomy. Otol Neurotol. 2009;30(8):1166–7.

25. Cremers CW, Beusen JM, Huygen PL. Hearing gain after stapedotomy, partial platinectomy, or total stapedectomy for otosclerosis. Ann Otol Rhinol Laryngol. 1991;100:959–61.

26. Spandow O, Soderberg O, Bohlin L.Long-term results in otosclerotic patients operated by stapedectomy and stapedotomy. Scand Audiol. 2000;29:186–90.

27. Persson P, Harder H, Magnuson B.Hearing results in otosclerosis surgery after partial stapedectomy, total stapedectomy and stapedotomy. Acta Otolaryngol. 1997;117:94–9.

28. Fisch U. Stapedectomy versus stapedectomy. Am J Otol. 1982;4:112–7.

29. Esquivel CR, Mamikoglu B, Wiet RJ. Long-term results of small fenestra stapedectomy compared with large fenestra technique. Laryngoscope. 2002;112:1338–41.

30. House HP, Hansen MR, Al Dakhail AAA, House JW. Stapedectomy versus stapedotomy: comparison of results with long-term follow-up. Laryngoscope. 2002;112:2046–50.

31. Wegner I, Kamalski DM, Tange RA, Vincent R, Stegeman I, van der Heijden GJ, Grolman W.Laser versus conventional fenestration in stapedotomy for otosclerosis: a systematic review. Laryngoscope. 2014;124(7):1687–93.

32. Yavuz H, Caylakli F, Ozer F, Ozluoglu LN.Reliability of microdrill stapedotomy: comparison with pick stapedotomy. Otol Neurotol. 2007;28(8):998–1001.

33. Rosowski JJ, Merchant SN.Mechanical and acoustic analysis of middle ear reconstruction. Am J Otol. 1995;16:486–97.

34. Huber AM, Ma F, Felix H, Linder T. Stapes prosthesis attachment: the effect of crimping on sound transfer in otosclerosis surgery. Laryngoscope. 2003;113:853–8.

35. Gierek T, Witkowska M, Zbrowska-Bielska D, Klimczak-Gołab L. Analysis of results of stapedotomy in patients with obliterative otosclerosis. Otolaryngol Pol. 2006;60(3):377–83.

36. Vincent R, Sperling NM, Oates J, Jindal M.Surgical ndings and long-term hearing results in 3050 Stapedotomies for primary otosclerosis: a prospective study with the otology-neurotology database. Otol Neurotol. 2006;27:S25–47.

37. Gersdorff M, Nouwen J, Gilain C, Decat M, Betsch C. Tinnitus and otsclerosis. Eur Arch Otorhinolaryngol. 2000;257:314–6.

38. Berling Holm K, Knutsson J, Strömbäck K, Danckwardt Lillieström N, Papatziamos G, Rosenblad A, Von Unge M. Taste disturbance after stapes surgery: an evaluation of frequency, severity, duration, and quality-of-life. Acta Otolaryngol. 2017;137(1):39–43.

39. Birch L, Elbrond O.Stapedectomy and vertigo. Clin Otolaryngol. 1985;10:217–23.

40. Mansour S, Magnan J, Haidar H, etal. Comprehensive and clinical anatomy of the middle ear. Berlin: Springer; 2013. p.49–81.

41. Glasscock ME III, Storper IS, Haynes DS, Bohrer PS. Twenty-ve years of experience with stapedectomy. Laryngoscope. 1995;105:899–904.

Congenital Hearing Loss

Данная книга находится в списке для перевода на русский язык сайта https://meduniver.com/

AbdulsalamAl-Qahtani, ZaidAltamimi, andReniK.Chandran

9.1 Introduction

Hearing loss present at birth (congenital hearing loss) is categorized according to the underlying pathology to hereditary (syndromic or nonsyndromic), non-hereditary, and idiopathic. Three types of hearing loss can be induced depending on the site of the lesion: conductive, sensorineural, or a combination of both (mixed) [1].

9.2 Neonatal Hearing Screening

Newborn hearing screening programs show that the incidence of congenital hearing loss is 2–4 children per 1000 births, and it is considered as the most frequently occurring birth defect in the US [2, 3]. Mutations in GJB2 account for 50%

A. Al-Qahtani (*) Hamad Medical Corporation, Doha, Qatar

Otology Clinical Fellow, Hamad Medical Corporation, Doha, Qatar e-mail: aalqahtani@hamad.qa

Z. Altamimi Otology Clinical Fellow, Hamad Medical Corporation, Doha, Qatar e-mail:

Zaltamimi@hamad.qa

R. K. Chandran Audiology, Hamad Medical Corporation, Doha, Qatar e-mail: RChandran1@hamad.qa

of people with severe-to-profound congenital autosomal- recessive non-syndromic hearing loss [4].

Neonatal hearing screening programs are available for the early detection of this condition. More than 50% of cases of permanent hearing impairment in childhood can be detected shortly after birth [5]. Using the 1-3-6 model intends to screen all newborns within the first month of birth for early diagnosis and subsequent early management with a better developmental outcome [6]. View the algorithm below to check the steps of the screening. However, passing the neonatal screening does not rule out hearing impairment in childhood. Progressive or late-onset hearing loss can be undetected by neonatal screening programs [6].

https://doi.org/10.1007/978-3-030-54088-3_9

AL GRAWANY

105

106

A. Al-Qahtani et al.

Neonatal screening

Pass

No Risk Factors

Discharge The Patient

Genetic

testing

Positive Risk Factors

Re-assessment 1 to 4

weeks

Pass

Evaluate for TORCH

infections

Fail

Confirmed Hearing Loss

9.3 Evaluation ofaChild withCongenital Hearing Loss

The general approach to evaluating the child with suspected congenital hearing loss includes the following:

• History: pregnancy history, perinatal and

postnatal period, NICU admission, postnatal infection, ototoxic medications, co-existing medical conditions, family history of hearing loss in rst- and second-degree relatives, consanguinity, and ethnic origin.

• Physical examination: a full head and neck

examination should be performed to including any dysmorphic features, the shape, and position of the external ears, neck examina-

Fail

Re-assessment 1 to 4 weeks

Pass

Audiology re-assessment

Imaging

studies

Fail

Normal Hearing

Screening other

anomalies

tion for cysts, sinuses, and scars (branchiooto-renal syndrome), swelling of the thyroid gland (may indicate Pendred syndrome), and unusual pigmentation of the hair, skin, or eyes (which may indicate an auditory pigmentary disorder).

• Investigation: the following should be con-

sidered: genetic testing, CT/MRI imaging, thyroid function tests, cardiology evaluation (Echo and ECG, for possible association with Jervell and Lange-Nielsen syndrome or congenital heart conditions), or ophthalmology assessment (electroretinogram if suspected Usher syndrome).

Risk factors for permanent congenital, delayed, or progressive hearing loss in childhood are described in Box 9.1 [5].

9 Congenital Hearing Loss

Данная книга находится в списке для перевода на русский язык сайта https://meduniver.com/

Box 9.1 Risk Factors for Permanent Congenital, Delayed or Progressive Hearing Loss in Childhood

• Hearing, speech, language, or developmental delay

•

Family history of hearing loss Neonatal intensive care unit stay >5days or

• receiving any of the following treatments: extra corporal membrane oxygenation, assisted ventilation, ototoxic drugs (e.g., gentamycin and tobramycin), loop diuretics, or exchange transfusion for hyperbilirubinemia

• In utero infections (toxoplasmosis, rubella, cytomegalovirus, herpes simplex, or syphilis)

•

Craniofacial anomalies, including ear tags, ear

pits, and anomalies that involve the outer ear, external auditory canal, and temporal bone

Physical ndings associated with a syndrome

• known to cause permanent hearing loss (e.g.,, white forelock)

•

Syndromes associated with congenital hearing

loss or progressive or late-onset hearing loss

Neurodegenerative disorders or sensorimotor

• neuropathies

•

Conrmed bacterial or viral meningitis

• Head trauma, especially of the basal skull, or temporal bone fractures that require hospitalization

Chemotherapy

•

9.4 Categories ofCongenital Hearing Loss

1. Idiopathic (25%).

2. Hereditary (50%):

• Non-syndromic (70%): more common,

the prex “DFN” to designate nonsyndromic DeaFNess, DFN followed by an A implies dominant inheritance, whereas B implies recessive inheritance and X implies X-linked inheritance. Autosomal recessive (AR) in (75–80%), GJ2B mutation (autosomal recessive) coding for the protein gap junction beta 2 (also called connexin 26) results in impaired Potassium (K+) exchange. Autosomal dominant (AD) in (20–25%), X-linked in (2–4%), and mitochondrial in <1% [5].

Syndromic (30%): Divided into AR, AD,

• and X-linked. The most common syndromic form of hereditary SNHL is Usher syndrome [7], and other AR syndromes include Pendred syndrome and Jervell and Lange–Nielsen Syndrome. The most common AD syndrome is Waardenburg [7], and others include Treacher–Collins Syndrome (Fig. 9.1), Branchio-oto-renal Syndrome (mainly mixed hearing loss), Neurobromatosis Type 2, and Stickler Syndrome. X-linked includes Alport’s syndrome. Box

9.2 describes the features of

the most common congenital hereditary hearing loss syndromes.

3. Non-hereditary (25%)

• Malformations: arrest in normal develop-

ment may result in hearing impairment depending on the timing and nature of the developmental insult. About 65% of such abnormalities are bilateral, and 35% are unilateral. Malformations include Membranous (Alexander’s Aplasia, Scheibe Deformity, and Siebenmann–Bing Dysplasia) and Osseous and Membranous (Cochlear Hypoplasia, Mondini, Common Cavity, Cochlear Aplasia, Michel Aplasia, and Small Internal Auditory Canal). Mondini malformation is the most common type of cochlear malformation [1].

– Alexander aplasia is one of the mem-

branous malformations, where cochlear duct differentiation at the level of the basal coil is limited with resultant effects on the organ of Corti and the ganglion cells. Hearing assessment shows high-frequency sensorineural hearing loss with adequate residual hearing in the low frequencies, and amplication devices can be used.

– Scheibe aplasia (cochleosaccular dys-

plasia or Pars Inferior dysplasia) is one of the membranous malformations, and it is a relatively common cochlear malformation. Scala media is compromised due to the failure of the organ of Corti development affecting the tectorial and the Reissner’s membranes. It is

107

AL GRAWANY

108

A. Al-Qahtani et al.

Fig. 9.1 Treacher–Collins syndrome

reported in Jervell and Lange Nielsen, Usher, and Waardenburg syndromes as well as in congenital rubella infants. Amplication devices with rehabilitative intervention are benecial in many of these children.

– Michel aplasia is one of the osseous

and membranous malformations, where there is complete agenesis of the petrous portion of the temporal bone resulting in absence of normal inner structures and therefore complete deafness. The developmental arrest occurs early prior to the end of the third gestational week.

– Mondini deformity is one of the osse-

ous and membranous malformations, and it is the most common type of cochlear malformation [1]. The developmental arrest occurs at the sixth week of gestation. It presents with an incomplete partition of the cochlea where it contains only about 1.5 turns with only

the basal coil and absence of the apical modiolus and interscalar septum. Mondini deformity is associated with enlarged vestibular aqueduct and dilated vestibule. It is seen in Pendred, Waardenburg, Treacher–Collins, and CHARGE syndromes, and it is also seen in congenital cytomegalovirus (CMV) infection.

– Enlarged Vestibular Aqueduct

Syndrome is dened as a vestibular aqueduct measuring more than 1.5mm on CT scan. It is thought to be one of the most common congenital causes of

sensorineural hearing loss. It presents

with early onset bilateral progressive sensorineural hearing loss. It is associated with Pendred syndrome, vestibular anomalies, Mondini malformation, and other cochlear anomalies. Mainstay of treatment is conservative management, including avoidance of head trauma and contact sports.

9 Congenital Hearing Loss

Данная книга находится в списке для перевода на русский язык сайта https://meduniver.com/

109

• TORCH Infections: It includes

Toxoplasmosis, Other (syphilis, varicellazoster, parvovirus B19), Rubella, Cytomegalovirus (CMV), and Herpes.

– Cytomegalovirus: the most com-

mon cause of congenital viral deafness [8].

– Mumps: the most common infectious

cause of acquired sensorineural hearing loss.

• Other perinatal factors: prematurity, low

birth weight, teratogenic drugs, maternal diabetes, hyperbilirubinemia, and neonatal sepsis.

Box 9.2 Overview of Congenital Hearing Loss Syndromes

Syndrome Features Comments Autosomal recessive Usher syndrome Sensorineural hearing loss, retinitis pigmentosa,

Pendred’s syndrome

Jervell and Lange-Nielsen syndrome Autosomal dominant Waardenburg syndrome

Treacher–Collins syndrome

Branchio-otorenal (BOR) syndrome

X-linked Alport’s syndrome

and vestibular symptoms Type 1, profound hearing loss, vestibular symptoms, and retinitis pigmentosa beginning in rst decade Type 2, stable mild to severe hearing loss and retinitis pigmentosa in rst to second decade Type 3, progressive hearing loss, variable vestibular symptoms, and variable onset of retinitis pigmentosa Sensorineural hearing loss (severe to profound), goiter, and abnormality of the bony labyrinth (Mondini dysplasia or enlarged vestibular aqueduct) Profound sensorineural hearing loss with prolongation of QT interval, syncopal episodes, and risk of sudden death

Most common type of AD; hair (white forelock

1), eyes (heterochromia iridis)

Type 1, white forelock and dystopia canthorum Type 2, dystopia absent Type 3, upper limb defects Type 4, increased incidence of Hirschsprung’s

disease Conductive hearing loss, underdevelopment of facial bones (malar and zygomatic hypoplasia) with malformed ossicles, microtia, cleft palate; micrognathia, downward slanting eyes, coloboma of the eyelid Hearing loss; preauricular pits; malformed pinnae; Branchial cysts or stulae; renal anomalies (structural malformations to agenesis)

Hematuria with progressive renal failure, progressive late-onset high-frequency sensorineural hearing loss; anterior lenticonus and macular ecks

Commonest cause of deafness with blindness, early diagnosis of retinitis pigmentosa possible with electroretinography, and cochlear implants effective

Iodine transport defect diagnosed by perchlorate discharge test in those homozygous for Pendred’s syndrome (SLC26A4) and euthyroid goiter Should have a thorough cardiac evaluation, carriers also at risk for sudden death, and treatment effective

Hearing loss caused by defective migration of pigment cells to stria vascularis

Results from mutations in 1 of 3 collagen genes expressed in glomerular basement membrane

AL GRAWANY

110

Take-Home Messages

• The most common prenatal cause of hearing loss is intrauterine infection (CMV).

• Mutations in GJB2 account for 50% of severe-to-profound autosomal-recessive non-syndromic deafness.

• The goal of newborn testing is to screen by 1 month of age, diagnose hearing loss by 3 months of age, and initiate intervention by 6months of age.

• Screening based on risk factors only detects 50% of infants with substantial congenital hearing loss.

References

1. Shibata SB, Eliot Shearer A, Richard JH. Genetic sensorineural hearing loss, Chap. 148. In: Cummings otolaryngology: head & neck surgery. 6th ed. Philadelphia: Elsevier; 2015. p.2285–300.

A. Al-Qahtani et al.

2. Parving A. The need for universal neonatal hearing screening–some aspects of epidemiology and identication. Acta Paediatr. 1999;88(432):69–72.

3. White KR. The current status of EHDI programs in the United States. Ment Retard Dev Disabil Res Rev. 2003;9(2):79–88.

4. Smith RJ, Robin NH. Genetic testing for deafness– GJB2 and SLC26A4 as causes of deafness. J Commun Disord. 2002;35(4):367–77.

5. Chi DH, Sabo DL.Pediatric audiology and implantable hearing device, Chap. 100. In: Bailey’s head and neck surgery: otolaryngology. 5th ed. Philadelphia: Lippincott Williams & Wilkins; 2014. p.1507–22.

6. Yoshinaga-Itano C, Sedey AL, Coulter DK, Mehl AL.Language of early- and later-identied children with hearing loss. Pediatrics. 1998;102:1161–71.

7. Smith RJH, Shearer AE, Hildebrand MS, et al. Deafness and hereditary hearing loss overview. 1999 Feb 14 [Updated 2014 Jan 9]. In: Pagon RA, Adam MP, Ardinger HH, et al., editors. GeneReviews® [Internet]. Seattle, WA: University of Washington, Seattle; 1993–2017.

8. Fowler KB, Boppana SB.Congenital cytomegalovirus (CMV) infection and hearing decit. J Clin Virol. 2006;35:226.

Sensorineural Hearing Loss (SNHL)

Данная книга находится в списке для перевода на русский язык сайта https://meduniver.com/

AishaLarem, ZaidAltamimi, andAdhamAljariri

10.1 Introduction

Hearing loss can be dened as an impairment in the ability to perceive sound stimulus. It includes three types: Conductive, Sensorineural, and Mixed hearing loss. Sensorineural hearing loss (SNHL) occurs due to defect in the sensory apparatus of the cochlea (sensory), or the peripheral pathways of conduction of nerve impulses (XIII CN) to the brain or in the central auditory pathway or cortex (neural).

Knowledge of growth, development, and functional anatomy of the inner ear is important to be able to identify the pathology and diagnose it. Membranous labyrinth develops from otic placode from the third week of the gestational age (GA) and then it becomes otocysts subdivided into vestibular and cochlear compartments. Cochlea reaches the shape of adult form at 10weeks and adult size at 20weeks GA.Cochlea is composed of a 35mm coiled bony tube with 2.5 turns, and it is divided into scala vestibuli, scala tympani, and scala media. Scala vestibuli and scala tympani contain perilymph, scala media is located between scala vestibuli and tympani, and

A. Larem (*) · Z. Altamimi · A. Aljariri Hamad Medical Corporation, Doha, Qatar e-mail: alarem@hamad.qa; ZAITamimi@hamad.qa;

AAljariri@hamad.qa

it contains the endolymph. Scala media is bounded by Reissner’s membrane, basilar membrane, osseous spiral lamina, and stria vascularis in the lateral wall, and it contains the organ of Corti. Organ of Corti is the receptor organ for hearing, composed of outer and inner hair cells, supporting cells, and the tectorial membrane. Inner and outer hair cells are differing in morphology and innervation. Inner hair cells’ neural innervation is by type I neurons which are predominantly afferent neurons, where 15–20 of these neurons innervate each inner hair cell. Outer hair cells’ neural synapses are by type II neurons which are predominantly efferent, where each type II neuron branches to innervate around ten outer hair cells. Sound transduction is dened as converting sound vibrations (mechanical energy) into neural impulse (electrical energy) by the movement of the basilar membrane with the hair cells against the tectorial membrane, stimulating generation of nerve ending impulses in spiral ganglion. The spiral ganglion sends axons into the cochlear nerve. The cochlear nerve transmits auditory information up a series of nuclei to the auditory cortex where perception occurs. These nuclei include: (1) cochlear nucleus, (2) superior olivary nuclei, (3) lateral lemniscus, (4) inferior colliculus, and (5) medial geniculate nuclei.

Sensorineural hearing loss (SNHL) is fea-

tured with the following

https://doi.org/10.1007/978-3-030-54088-3_10

AL GRAWANY

111

<<< < Предыдущая 1 2 3 4 5 6 7 8 9 10 1112 / 5012 13 14 15 16 17 18 19 20 21 22 23 24 25 > Следующая >>>

Соседние файлы в папке Библиотека им академика М.И. Перельмана

#
30.08.202651 Мб0Книга_4440_Библиотеки_им_академика_М_И_Перельмана.pdf
#
30.08.202650 Мб0Книга_4442_Библиотеки_им_академика_М_И_Перельмана.pdf
#
30.08.202649 Мб0Книга_4443_Библиотеки_им_академика_М_И_Перельмана.pdf
#
30.08.202649 Мб0Книга_4444_Библиотеки_им_академика_М_И_Перельмана.pdf
#
30.08.202649 Мб0Книга_4445_Библиотеки_им_академика_М_И_Перельмана.pdf
#
30.08.202649 Мб0Книга_4446_Библиотеки_им_академика_М_И_Перельмана.pdf
#
30.08.202648 Мб0Книга_4449_Библиотеки_им_академика_М_И_Перельмана.pdf
#
30.08.202648 Мб0Книга_4450_Библиотеки_им_академика_М_И_Перельмана.pdf
#
30.08.202648 Мб0Книга_4451_Библиотеки_им_академика_М_И_Перельмана.pdf
#
30.08.202647 Мб0Книга_4452_Библиотеки_им_академика_М_И_Перельмана.pdf
#
30.08.202647 Мб0Книга_4453_Библиотеки_им_академика_М_И_Перельмана.pdf