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250
B. Celikgun et al.
remote microphone and TV adapter may be recommended to minimize the patient’s
problem with understanding speech in noise (Fig.11.8).
11.10.3 Case 3
Case 3 has a bilateral symmetric profound sensorineural hearing loss. In addition,
the patient had a bilateral type A tympanogram and the patient’s acoustic reexes
could not be obtained. Looking at the case information, the rst thing that stands out
is that the patient has very poor speech discrimination skills. Hearing aid models
that can be used for severe/profound hearing loss are limited. The BTE Superpower
or BTE Ultra Power hearing aids released by the manufacturers are the rst options
that come to mind for severe to profound hearing loss. Especially for candidates
who do not have sufcient speech discrimination skills, it is recommended to use a
remote microphone accessory in addition to the hearing aid to increase the SNR
ratio. If the medical and psychological condition of the candidate is appropriate,
cochlear implant surgery may also be considered (Fig.11.9).
Fig. 11.8 Case study 2.
SRTs (Speech Reception
Thresholds); SDSs (Speech
Discrimination Scores);
UCLs (Uncomfortable
Levels)
11 Selection and Application Principles of Hearing Aids in Pediatric and Adult…
Fig. 11.9 Case study 3.
SRTs (Speech Reception
Thresholds); SDSs (Speech
Discrimination Scores);
UCLs (Uncomfortable
Levels)
251
11.10.4 Case 4
In this case, the patient has a moderate sensorineural hearing loss that increases
toward the high-frequencies in the right ear and a profound mixed hearing loss in
the left ear. The air-bone gap in the patient’s left ear was found to be inconsistent
with the left ear speech discrimination results. In this case, bone conduction masks
can be retested for verication. Although the discrimination score in the left ear is
0%, making “binaural” hearing difcult, it may still be recommended to provide
separate amplication in each ear. Another hearing aid option is the BiCROS hearing aid. In this system, the hearing aid in the patient’s right ear both strengthens the
hearing in the right ear and receives the sound from the transmitter in the left ear.
This patient is not a candidate for BAHS surgery, which can be performed for asymmetrical hearing loss, because the bone conduction thresholds of the right ear are
not sufcient. However, a combination of a hearing aid in the right ear and a cochlear
implant in the left ear may be preferred. Finally, in cases of asymmetrical hearing
loss, if both ears cannot be effectively amplied, additional wireless accessories
such as a remote microphone and TV adapter may be recommended to minimize the
patient’s problem with understanding speech in noise (Fig.11.10).
252
Fig. 11.10 Case study 4.
SRTs (Speech Reception
Thresholds); SDSs (Speech
Discrimination Scores);
UCLs (Uncomfortable
Levels)
B. Celikgun et al.
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Part III
Outer Ear Pathologies and Management
Auricula Anomalies andAuricula Atresia
12
ZeynelÖztürk, OguzhanOguz, andNurayBayarMuluk
12.1 Introduction
The external auditory canal (EAC) and middle ear components do not fully mature
in congenital aural atresia (CAA). The ear canal and middle ear embryonic development can be halted at any stage. Consequently, the severity of this deformity might
uctuate, which can be challenging for the clinician. In extreme cases, the ear canal
is not discernible (total atresia), and the middle ear and its components (ossicles or
ear bones) are either completely missing or severely underdeveloped. A shallow
blind pouch may form at the end of the ear canal if there is any sign of an external
auditory meatus (outer opening). A stenotic (narrow) ear canal, a tiny hole that leads
into the middle ear canal, and even a primitive tympanic membrane can be seen in
milder cases. The ossicular chain, which connects the tympanic membrane to the
ear bones, might or might not be present in these ears [1].
One of the most challenging procedures an ear specialist encounters is surgery to
x CAA.A surgically lined external auditory canal free of debris and moisture is
one of the primary aims of the procedure, along with the patient’s permanent hearing loss reduction or improvement [1].
Z. Öztürk
Faculty of Medicine, Department of Otorhinolaryngology, Nişantaşı University,
Istanbul, Turkey
Baypark Hospital, Istanbul, Turkey
O. Oguz
Department of Audiology, Health Services Vocational School, Istanbul Nişantaşı University,
Istanbul, Turkey
Dr. Oğuzhan Oğuz Wellnose Clinic, Istanbul, Turkey
N. BayarMuluk (*)
Faculty of Medicine, Department of Otorhinolaryngology, Kirikkale University,
Kirikkale, Turkey
© The Author(s), under exclusive license to Springer Nature
Switzerland AG 2024
M. T. Kalcioglu et al. (eds.), Otology Updates, Comprehensive ENT,
https://doi.org/10.1007/978-3-031-76173-7_12
259
260
Z. Öztürk et al.
Additionally challenging for surgical reconstruction is the presence of microtia,
also known as “small ear,” or inadequate development of the auricle (pinna), which
is a common complication of CAA [2]. There are three classical grades of microtia:
It is a smaller-than-average ear with all the typical ridges and valleys of the fragile
cartilage; II is a more severe case. II is smaller than average, and the cartilage does
not fully grow in its ridges and valleys. A “peanut” ear, characterized by a tiny remnant of cartilage or soft tissue, is grade III microtia. The condition is called anotia
without an auricle and any other ear cartilage or skin attachment [1].
Though they develop independently in the embryo, the ear canal and auricle
frequently follow the same patterns as the middle ear structures, with a more developed auricle typically accompanied by a more developed middle ear space and
ossicular chain [3].
12.2 Embryology oftheEar
At 24days of gestation, the embryo’s lateral surface begins to show a sequence of
ectodermal outpouchings (arches) and indentations (clefts/grooves), which develop
into the ear canal and middle ear. This structure is called the branchial apparatus.
After the primary meatus (the rst branchial groove) invaginates, the ear canal can
start to take shape. Positioned between the rst branchial arch rostrally (facing the
head) and the second branchial arch caudally (facing the tail) [1] is this region.
Even before the second trimester, the branchial groove invades and moves medially as an epithelial plate. The rst pharyngeal pouch’s lateral expansion is momentarily met by its ingrowth. The endoderm of the foregut (alimentary canal) gives rise
to the rst pharyngeal pouch, which in turn gives rise to the middle ear cleft and the
Eustachian tube. Tympanic membrane precursor, the metal plate, is formed when
the ingrowth of the rst pharyngeal pouch and rst branchial groove joins. Around
the sixth month of gestation, the epithelial scale starts to open, or canalize, from the
inside outward, meeting the major meatus.
The ear canal comprises a cartilaginous part on one side and a bony tympanic
ring on the other when the baby is born. As a baby is born, the bony tympanic ring
grows longer and changes shape, becoming a bony cylinder. So, when a child is four
or ve, their ear canal has grown to adult size [1].
The ear’s maturation might halt at any stage in congenital amaurosis. Total atresia happens if the process is stopped before the ear canal is canalized. The extent to
which the external ear canal is permeable changes as a function of the amount of
development inhibited during canalization [1].
Beginning with the branchial apparatus, the ear canal develops in tandem with
the middle ear cavity and ear bones (ossicles). The rst pharyngeal pouch develops
laterally, creating the space between the ears. The ossicles are nally encased in the
middle ear and integrated into that region. The ossicles originate from the Reichert
cartilage in the rst branchial arch and the Meckel cartilage in the second branchial
arch. If the ear canal does not fully mature, it will have a knock-on effect on the
middle ear, causing it to be underdeveloped and with a narrowed space. Common