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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_4518_Библиотеки_им_академика_М_И_Перельмана
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• Management:
– NPO, IV uids; do not induce vomiting.
– Reux management.
– Empiric antibiotics debated.
– Esophagoscopy at 24–72h.
Consider intraoperative nasogastric tube placement (stent)
Increased risk of iatrogenic perforation after 72h
– Grading:
Grade 1: mucosal erythema
Grade 2: mucosal erythema with non-circumferential ulceration
Grade 3: circumferential ulceration
Grade 4: circumferential ulceration with perforation
– Observation for Grade 1–2; consider steroids.
– More aggressive management for Grade 3–4, may require esophagectomy.
– Sequelae: esophageal stricture requiring repeat dilations, esophageal perfora-
tion, and mediastinitis.
A. F. Karas and J. W. Lee
Esophageal Foreign Bodies
• Anatomical structures compressing the esophagus, points of potential location
for foreign bodies:
– Cricopharyngeus
– Aortic arch
– Left mainstem bronchus
– Lower esophageal sphincter
• Some small foreign bodies can be followed by serial X-rays to ensure passage;
observation for 24h.
• Batteries (“double-halo sign”) and sharp objects may lead to catastrophic complications and mortality and should be promptly removed.
– For battery, frequent ingestion of honey (10mL every 10min) while en route
to the ER may be protective but should not delay care. Can only be done in
children ≥12months
• Food impaction:
– Consider evaluation for esophageal stricture, peristaltic dysfunction, biopsy
for eosinophilic esophagitis (>15 eosinophils per high-power eld), and/or
signicant personal or family history of atopy
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Further Reading
Ho AS, Koltai PJ.Pediatric tracheal stenosis. Otolaryngol Clin N Am. 2008;41(5):999–1021.
Kakodkar KA, Schroeder JW Jr, Holinger LD. Laryngeal development and anatomy. Adv
Otorhinolaryngol. 2012;73:1–11.
O-Lee TJ, Messner A. Subglottic hemangioma. Otolaryngol Clin N Am. 2008;41(5):903–11,
viii–ix.
Rahbar R, Rouillon I, Roger G, Lin A, Nuss RC, Denoyelle F, et al. The presentation and
management of laryngeal cleft: a 10-year experience. Arch Otolaryngol Head Neck Surg.
2006;132(12):1335–41.
King EF, Blumin JH.Vocal cord paralysis in children. Curr Opin Otolaryngol Head Neck Surg.
2009;17(6):483–7.
Wiatrak BJ.Overview of recurrent respiratory papillomatosis. Curr Opin Otolaryngol Head Neck
Surg. 2003;11(6):433–41.
Derkay CS, Malis DJ, Zalzal G, Wiatrak BJ, Kashima HK, Coltrera MD.A staging system for
assessing severity of disease and response to therapy in recurrent respiratory papillomatosis.
Laryngoscope. 1998;108(6):935–7.
Lawlor CM, Dombrowski ND, Nuss RC, Ranbar R, Choi SS.Laryngeal web in the pediatric
population: evaluation and management. Otolaryngol Head Neck Surg. 2020;162(2):234–40.
Shah RK, Mora BN, Bacha E, Sena LM, Buonomo C, Del Nido P, etal. The presentation and
management of vascular rings: an otolaryngology perspective. Int J Pediatr Otorhinolaryngol.
2007;71(1):57–62.
Kay M, Wyllie R.Caustic ingestions in children. Curr Opin Pediatr. 2009;21(5):651–4.
Marom T, Goldfarb A, Russo E, Roth Y.Battery ingestion in children. Int J Pediatr Otorhinolaryngol.
2010;74(8):849–54.
Anfang RR, Jatana KR, Linn RL, Rhoades K, Fry J, Jacobs IN. pH-neutralizing esophageal irriga-
tions as a novel mitigation strategy for button battery injury. Laryngoscope. 2019;129(1):49–57.
Chapter 5
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Pediatric Otology andCochlear Implants
AnatoliF.Karas andJanetWaiminLee
Pearls
• The workup for congenital sensorineural hearing loss (SNHL) should consist of
at least an electrocardiogram (EKG), ophthalmologic evaluation, and an imaging
study (MRI/CT).
– MRI can be obtained early on when infant is very young, and infant may sleep
through study without the need for sedation.
– Eye exam is important if patient has at least one special sensory impairment.
– Genetics evaluation may be useful for counseling and prognostic information.
– Other studies can be obtained as dictated by individual cases, but “shotgun”
approach to testing may be expensive and unnecessary.
• Aural polyps in the external auditory canal (EAC) should trigger suspicion for an
associated underlying cause for exuberant inammatory/granulation tissue
reaction.
– Prior to biopsy of external auditory canal lesion, imaging should be consid-
ered—rule out vascular or intracranial connection.
• Steroids may be protective for SNHL in cases of meningitis; however, early
cochlear implantation in patients with signicant SNHL from meningitis is
important due to labyrinthitis ossicans.
A. F. Karas
Department of Otorhinolaryngology, Head & Neck Surgery, Rush University Medical Center,
Chicago, IL, USA
Department of Pediatrics, Rush University Medical Center, Chicago, IL, USA
e-mail: anatoli_f_karas@rush.edu
J. W. Lee (*)
Department of Head and Neck Surgery & Communications Sciences, Duke University
Medical Center, Durham, NC, USA
e-mail: janet.w.lee@duke.edu
© Springer Nature Switzerland AG 2023
F. Y. Lin, Z. M. Patel (eds.), ENT Board Prep,
https://doi.org/10.1007/978-3-031-26048-3_5
45
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• The importance of binaural hearing is becoming more accepted—hearing in
noise, summation, squelch, and directionality.
– Bilateral cochlear implants demonstrate some improved objective measures.
A. F. Karas and J. W. Lee
External Auditory Canal Pathology
• Otitis externa
– Pseudomonas, Staphylococcus aureus, can be polymicrobial
– Treatment: aural hygiene, debridement, ototopical antibiotic and steroid
drops, may require wick placement if edema
• Otomycosis
– Fungal infection of EAC skin, often related to ototopical antibiotics/steroid
use, can also be seen in immunocompromised populations
– Aspergillus, Candida most common
– Fungal debris in EAC, often with hyphae and sometimes spores on
otomicroscopy
– Treatment: debridement, re-acidication of canal, topical antifungals
• Aural polyps—granulation tissue formation which may be associated with any
of the following:
– Chronic suppurative otitis media, with or without cholesteatoma
– Underlying tympanostomy tube
– Malignant otitis externa (necrotizing otitis externa, temporal bone
osteomyelitis)
Susceptible populations
• Poorly controlled diabetics
• Immunosuppressed—leukemia, bone marrow transplant, organ transplant, etc.
TC-99 imaging helpful for initial diagnosis but stays positive after resolution; Gallium-67 and Indium-111 scans better for disease monitoring
– Malignancies
Rhabdomyosarcoma—most common pediatric temporal bone malignancy
Nonrhabdomyosarcomatous sarcoma—extremely rare, includes chondrosarcoma, chordoma, osteosarcoma, Ewing sarcoma, chloroma
Lymphoma and carcinoma—very rare
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– Langerhans cell histiocytosis (pseudomalignancy)
Rare locally destructive condition from proliferation of Langerhans cells
Eosinophilic granuloma (unifocal), Hand-Schuller-Christian disease (unisystem multifocal), Letterer-Siwe disease (multisystem)
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Otitis Media (OM)
• Shorter, narrower, more horizontal eustachian tube makes children uniquely
prone to chronic otitis media with effusion (COME) and acute otitis media (AOM)
• Risk factors: gender (COME more common in males), bottle feeding, sibling
history of OM, daycare attendance, lower socioeconomic status, passive smoke
exposure, craniofacial abnormalities, immunodeciency
• Etiologic agents of AOM: Streptococcus pneumoniae, Haemophilus inuenzae,
Moraxella catarrhalis, Group A Strep
• Sequelae: adhesive otitis media, chronic suppurative otitis media, tympanic
membrane perforation, tympanosclerosis, tympanic membrane retraction pocket,
cholesteatoma, ossicular necrosis, hearing loss, mastoiditis
– Adenoidectomy may help with eustachian tube dysfunction and help prevent
need for replacement of tympanostomy tubes and other sequelae of COME/
AOM, currently recommended as possible adjunct to tympanostomy tube
placement for COME in patients ≥4years old
• Acute indications for myringotomy±tympanostomy tube placement:
– AOM in a seriously ill or toxic child, AOM unresponsive to antibiotics, sup-
purative complications of AOM, AOM in newborn or immunocompromised
patient, AOM with facial paralysis, AOM with meningitis or other intracranial process
• Indications for tympanostomy tube placement:
– Recurrent AOM (≥3 episodes in 6months, ≥4 episodes in 12months with at
least 1 episode in the past 6months)
– Bilateral COME ≥3months, unilateral COME ≥6months; earlier for signi-
cant (>25dB) hearing loss, speech/language delay, severe retraction pocket,
disequilibrium/vertigo, tinnitus
• Complications of tympanostomy tube placement:
– Otorrhea, granulation tissue, myringosclerosis, tympanic membrane perfora-
tion, cholesteatoma, early extrusion, plugged tube, loss of tube in middle ear,
retained tube
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A. F. Karas and J. W. Lee
Mastoiditis
• Complication of acute otitis media
• Based on clinical evaluation and history
– Acute AOM or within last 2weeks, otalgia, fever, protrusion of ear; redness,
pain, swelling behind ear
– ± otorrhea, conductive hearing loss (CHL)
• Intracranial complications
– Imaging (CT) to assess for bony coalescence
– Meningitis, subperiosteal abscess, subdural empyema, focal otitis encephali-
tis, brain abscess, lateral sinus thrombosis
– Symptoms include persistent headaches, lethargy, malaise, nausea/vomiting,
fever, severe otalgia; ataxia, blurred vision, CN/facial nerve decits, focal
seizures
• Treatment
– IV antibiotics, tympanostomy tube placement, I&D of subperiosteal abscess
if present, mastoidectomy
– Neurosurgical interventional for abscess/empyema
Congenital Sensorineural Hearing Loss
• 50% acquired, 50% hereditary
• 1/3 of hereditary cases are syndromic, 2/3 non-syndromic
• 80% of non-syndromic cases are autosomal recessive (AR), 15% autosomal
dominant (AD), 5% X-linked/mitochondrial inheritance
• Cytomegalovirus (CMV)
– Most common cause of non-hereditary SNHL in childhood.
– Most children asymptomatic at birth but can develop late sequelae; SNHL is
the most common sequela.
– Overall rate of HL in CMV infection ~12%.
• Auditory brainstem response (ABR)
– Newborn ABR waves I/II (eight nerve), III (olivary complex), V (inferior
colliculus)
• Otoacoustic emissions (OAEs)
– Spontaneous—of little clinical use, not present in all patients
– Evoked—transient evoked OAEs (TEOAE), distortion product
OAEs (DPOAE)
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• Continuing campaign for universal newborn hearing screening using
ABR or OAEs
– Referral for formal audiometric testing/sedated ABR if failure
• Auditory testing
– Behavioral observational audiometry: 0–6months
– Visual reinforcement audiometry: 6months to 2years
– Conditioned play audiometry: 2–5years
• Workup considerations
– EKG
– Ophthalmology consult—SNHL may be associated with visual problems and
want to maximize sensory input
– Imaging—indicated for evaluation for cochlear implantation, in children with
recurrent meningitis, history of sudden or progressive SNHL especially following head trauma, or where imaging results will impact patient counseling
CT
• Advantages
– Lower cost
– Increased availability
– Speed resolution of capturing images (may be able to avoid sedation)
– Better visualization of bony labyrinth and osseous, inner structures
• Disadvantages
– Radiation exposure
– Difcult to assess the presence of cochlear nerve
MRI
• Advantages
– Superior visualization of membranous labyrinth, internal auditory
canal (can visualize cochlear nerve), high T2 intensity
– Lack of ionizing radiation
• Disadvantages
– More expensive
– Increased length of time
– May require sedation due to length of exam
– Availability
– Genetics consultation—counseling and testing
Nonsyndromic: 50% AR caused by mutation at DNFB1 locus.
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A. F. Karas and J. W. Lee
• Connexin 26: product of gene GJB2; present in 30% of non-syndromic
congenital HL; most common mutation in Caucasians is 35delG.
• Connexin 30: product of GJB6.
Syndromic, Autosomal Dominant.
• Waardenburg: most common cause of syndromic AD hearing loss.
Multiple types, all with white forelock, heterochromia iridis or hypoplastic blue irides. Multiple genes including PAX3, MITF, EDNRB,
EDN3, and SOX10.
• Branchio-oto-renal: Second most common cause of syndromic AD
hearing loss. Can have SNHL, CHL, or mixed. Branchial cleft anomalies, ear anomalies (including preauricular pits), renal anomalies. Can
have ossicular and/or inner ear anomalies. Mutations in EYA1.
• CHARGE: coloboma, heart defects, choanal atresia, growth retardation,
genital abnormalities, ear abnormalities. Mutations in CHD7.
Syndromic, autosomal recessive.
• Usher: most common cause of syndromic AR hearing loss, most common cause of deaf-blindness. Variable onset of SNHL and retinitis pigmentosa depending on type. Multiple genes including USH1C, USH2A,
USH3A, MYO7A, SANS, CDH23, PCHD15, VLGR1, and WHRN.
• Pendred: Second most common cause of syndromic AR hearing loss.
Enlarged vestibular aqueduct, euthyroid goiter, cochlear dysplasia.
Associated with SLC26A4, FOXI1, and KCNJ10.
• Jarvell-Lange-Nielsen: Third most common cause of syndromic AR
hearing loss. Long QT, mutations in KCNQ1 and KCNE1.
Syndromic, variable or other inheritance patterns.
• Stickler: most commonly AD but can be AR.Progressive SNHL, cleft
palate, eye abnormalities. Most commonly COL2A1; multiple other collagen genes can be involved.
• Alport: usually X-linked, can also be AD or AR.Progressive SNHL and
glomerulonephritis, can also have eye ndings. Mutations in COL4A3,
COL4A4, and COL4A5.
– Other possible testing
CMV
Urinalysis
BMP/CBC
Thyroid function tests
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Congenital Inner Ear Malformations
• Membranous (~80%)
– Not visible using current imaging modalities
– Scheibe dysplasia: cochleosaccular aplasia, most common
– Bing-Siebenmann dysplasia: membranous aplasia of both cochlear and ves-
tibular portions, bony labyrinth normal
– Alexander aplasia: cochlear duct aplasia, especially affecting basal turn
– Associated with Jarvell-Lange-Nielsen, Refsum, Usher and Waardenburg
syndromes
• Bony and membranous (~20%)
– Michel (complete labyrinthine) aplasia: third week gestation
– Common cavity: fourth week
– Cochlear aplasia: fth week
– Mondini malformation (incomplete cochlear partitions): seventh week
Associated with Pendred, branchio-oto-renal, Waardenburg, Treacher
Collins, Wildervanck (Klippel-Feil), and Mobius syndromes
• Labyrinthine anomalies
– Present in 40% of patients with radiographically visible cochlear anomalies.
– Dysplasia more common that aplasia.
– Lateral semicircular canal develops last and is most commonly affected.
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• Aqueductal anomalies
– Enlarged vestibular aqueduct: >1.5mm in diameter; associated with Mondini
malformation, Pendred syndrome; ~40% of patients will progress to profound
SNHL classically precipitated by head trauma.
– Enlarged cochlear aqueduct: normal ~3–4 diameter.
• Internal auditory canal anomalies
– Narrow (<3mm) associated with absent or hypoplastic CN VIII
Cochlear Implant Criteria
• Age 12months or greater (evaluation can begin sooner).
• Bilateral severe to profound hearing loss.
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• Limited benet from hearing aids (usually 3–6-month trial).
• Strong family commitment.
• Educational plan and resources that emphasize the development of auditory skills.
• Prognosis of speech development will depend on age of implantation and if pre-/
post-lingual.
• Note that implantation may occur earlier and without trial of hearing aids in the
setting of progressive post-meningitic cochlear ossication.
• Special considerations: narrow IAC, cochlear nerve aplasia, and Michel aplasia.
Implantation can be considered, but appropriate counseling should be performed.
Also beware of perilymph gusher.
• FDA approvals are constantly changing.
A. F. Karas and J. W. Lee
Central Auditory Processing Disorder
• Auditory neuropathy spectrum disorder (ANSD)
– Normal OAEs
– Absent or abnormal ABR
– Suggests CN VIII dysfunction
– MRI and genetic workup recommended
– Variable benet with cochlear implantation
Conductive Hearing Loss (CHL)
• Bone conduction hearing aids
– Atresia/microtia
– Chronic otorrhea or abnormally small EAC where air conduction hearing aids
are insufcient
• Ossicular chain reconstruction if CHL following cholesteatoma resection necessitating removal of ossicles
• Atresiaplasty only in carefully selected patients according to Jahrsdoerfer criteria
– 1 point each for normal external ear, pneumatized middle ear, pneumatized
mastoid, normal facial nerve, malleus-incus complex, incus-stapes complex,
patent round window, and patent oval window.
– 2 points for present stapes.
– Traditionally, atresiaplasty can be considered in patient with score ≥6.
– Presence of stapes is probably most important single factor for prognosis of
good hearing outcome (closure of air-bone gap to <20dB).
• Middle ear implants may be more available in the future; indications/applications still under review
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