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Congenital Hearing Loss and Inner Ear Malformations
Built from Haynes-Glasscock-Shambaugh Surgery of the Ear, Seventh Edition
What’s inside
17 sections · 110 slides
Scope and Burden
- Prevalence of congenital hearing loss
- Genetic versus non-genetic aetiology
- Syndromic versus nonsyndromic genetic loss
- Treatment options in congenital hearing loss
Nonsyndromic Genetic Hearing Loss
- Inheritance patterns in nonsyndromic SNHL
- DFN nomenclature for nonsyndromic genes
- Autosomal recessive nonsyndromic hearing loss
- GJB2 and connexin 26
- SLC26A4 and Pendred syndrome
- Autosomal dominant nonsyndromic hearing loss
Syndromic Hearing Loss
- Diagnosis of the common hearing loss syndromes
- Usher syndrome phenotypes
- Pendred syndrome
- X-linked deafness with stapes gusher
- Common congenital hearing loss syndromes and associated features
- Inner ear anomalies by syndrome
- Systemic features by syndrome
Clinical Evaluation
- Directed history and physical examination
- Cytomegalovirus testing in neonates
- Imaging strategy in congenital SNHL
- Workup pathway in congenital SNHL
- Indications for GJB2 sequencing
- Comprehensive and whole exome testing
- Counselling before genetic testing
Congenital Inner Ear Anomalies
- Two groups of congenital inner ear anomaly
- Why bony labyrinthine anomalies matter
Jackler Classification
- Basis of the Jackler classification
- Anomalies in the Jackler classification
- Description of cochleovestibular anomalies as classified by Jackler et al.
- Anatomic description of the cochleovestibular anomalies
- Anatomic description of the cochleovestibular anomalies (continued)
- Complete labyrinthine aplasia on axial CT
- Aplasia versus labyrinthitis ossificans
- Common cavity malformation on axial CT
- Cochlear aplasia on axial CT
- Cochlear hypoplasia on CT and T2-weighted MRI
Phelps Classification
- Basis of the Phelps classification
- Categories in the Phelps classification
Sennaroglu Classification
- Basis of the Sennaroglu classification
- Sequence of the Sennaroglu classification
- Incomplete partition type I on CT and MRI
- Incomplete partition type II on CT and MRI
- Definition of the Mondini deformity
- Incomplete partition type III on CT and MRI
- Limitations of the developmental arrest model
Enlarged Vestibular Aqueduct
- Enlarged vestibular aqueduct on CT and MRI
- Diagnostic criteria for enlarged vestibular aqueduct
- Imaging modality choice in EVA
- Natural history and associations of EVA
Membranous Labyrinthine Anomalies
- Membranous labyrinthine anomalies
- Membranous labyrinthine anomalies and hearing loss pattern
- Anatomic description of membranous labyrinthine anomalies
Cochlear Nerve Anomalies
- Cochlear nerve deficiency on oblique sagittal MRI
- Definitions in cochlear nerve deficiency
- Indications for MRI of the cochlear nerve
- Implant candidacy in cochlear nerve deficiency
Implications for Cochlear Implantation
- Evolution of candidacy in malformed ears
- Core surgical difficulties in malformed ears
- Facial nerve anomalies in cochleovestibular malformation
- Aberrant facial nerve courses encountered
- Intraoperative management of the aberrant nerve
Surgical Approaches
- Surgical approaches to the malformed cochlea
- Transmastoid posterior tympanotomy approach
- Subtotal petrosectomy with blind sac closure
- Transmastoid retrofacial approach
- Suprameatal approach
- Transmastoid labyrinthotomy approach
Electrode Insertion and CSF Leak
- Mechanism of CSF gusher at cochleostomy
- Reported rates of CSF leak
- Stepwise management of intraoperative CSF leak
- Adjuncts and postoperative advice after a gusher
Electrode Selection and Imaging
- Principles of electrode array selection
- Array choice by malformation
- Electrode insertion into the internal auditory canal
- Intraoperative imaging to confirm array position
Audiologic Outcomes
- Overall speech perception after implantation
- Outcomes in the IP-EVA spectrum
- Outcomes in severe cochleovestibular dysplasia
- Outcomes in cochlear nerve deficiency
- Communication counselling in cochlear nerve deficiency
Summary
- Key points on aetiology and evaluation
- Key points on malformations and implantation
- References
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- Haynes-Glasscock-Shambaugh Surgery of the Ear, Seventh Edition
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