Title: L-191 (Unaffected)
Chapter: Developmental Defects
Chapter section: Non-Syndromic Hearing Loss / DFNA 9
TB Case Number: 231
Comments: Middle turn of cochlea
Gender: Male
Age(yrs.): 56
Otologic Dx: 1. Temporal bone, normal, left
2. Membranous labyrinth, normal, left
3. Bony labyrinth, normal, left
4. Artifact, removal, amputation, cochlea, partial, left
5. Artifact, manipulative intentional infracture of footplate, left
6. Artifact, bone dust, saw-g

Title: DFNA9, R-101
Chapter: Developmental Defects
Chapter section: Non-Syndromic Hearing Loss / DFNA 9
TB Case Number: 398
Comments: DFNA 9 with COCH mutation, middle turn
Gender: Male
Age(yrs.): 81
Otologic Dx: 1. Atrophy, collapse and distortion of the vestibular membranous labyrinth, bilateral, probably caused by the effects of aging
2. Degeneration of the vestibular nerves, bilateral, associated with or secondary to:
a. Presbycusis, neural type
b. Tympanosc

Title: DFNA9, L-280
Chapter: Developmental Defects
Chapter section: Non-Syndromic Hearing Loss / DFNA 9
TB Case Number: 230
Comments: DFNA 9 with COCH mutation, middle turn
Gender: Female
Age(yrs.): 86
Otologic Dx: 1. Hereditary deafness, sensorineural, severe, adult-onset, autosomal dominant, bilateral
2. Deposit, ground substance, proliferation, auditory and vestibular sense organs, bilateral
3. Deposit, glycosaminoglycans, auditory and vestibular sense organs

Title: L-191-2 (Unaffected)
Chapter: Developmental Defects
Chapter section: Non-Syndromic Hearing Loss / DFNA 9
TB Case Number: 231
Comments: Basal turn
Gender: Male
Age(yrs.): 56
Otologic Dx: 1. Temporal bone, normal, left
2. Membranous labyrinth, normal, left
3. Bony labyrinth, normal, left
4. Artifact, removal, amputation, cochlea, partial, left
5. Artifact, manipulative intentional infracture of footplate, left
6. Artifact, bone dust, saw-g

Title: DFNA9, L-270
Chapter: Developmental Defects
Chapter section: Non-Syndromic Hearing Loss / DFNA 9
TB Case Number: 230
Comments: DFNA 9 with COCH mutation, basal turn
Gender: Female
Age(yrs.): 86
Otologic Dx: 1. Hereditary deafness, sensorineural, severe, adult-onset, autosomal dominant, bilateral
2. Deposit, ground substance, proliferation, auditory and vestibular sense organs, bilateral
3. Deposit, glycosaminoglycans, auditory and vestibular sense organs

Title: L-201 (unaffected)
Chapter: Developmental Defects
Chapter section: Non-Syndromic Hearing Loss / DFNA 9
TB Case Number: 231
Gender: Male
Age(yrs.): 56
Otologic Dx: 1. Temporal bone, normal, left
2. Membranous labyrinth, normal, left
3. Bony labyrinth, normal, left
4. Artifact, removal, amputation, cochlea, partial, left
5. Artifact, manipulative intentional infracture of footplate, left
6. Artifact, bone dust, saw-g

Title: L-71 (unaffected)
Chapter: Developmental Defects
Chapter section: Non-Syndromic Hearing Loss / DFNA 9
TB Case Number: 231
Comments: Lateral ampulla
Gender: Male
Age(yrs.): 56
Otologic Dx: 1. Temporal bone, normal, left
2. Membranous labyrinth, normal, left
3. Bony labyrinth, normal, left
4. Artifact, removal, amputation, cochlea, partial, left
5. Artifact, manipulative intentional infracture of footplate, left
6. Artifact, bone dust, saw-g

Title: DFNA9, L-61
Chapter: Developmental Defects
Chapter section: Non-Syndromic Hearing Loss / DFNA 9
TB Case Number: 399
Comments: DFNA 9 with COCH mutation, Lateral ampulla
Gender: Male
Age(yrs.): 81
Otologic Dx: 1. Arachnoid cyst, internal auditory canal
2. Degeneration, vestibular sense organs, severe
3. Atrophic degenerative changes in structures of the cochlear duct
4. Severe degeneration, vestibular nerves
5. Degeneration of the cochlear nerve
6. Otosclerosis

Title: DFNA9, R-201
Chapter: Developmental Defects
Chapter section: Non-Syndromic Hearing Loss / DFNA 9
TB Case Number: 736
Comments: DFNA 9 with COCH mutation, basal turn of cochlea
Gender: Female
Age(yrs.): 59
Otologic Dx: 1. Hereditary sensorineural deafness, severe, bilateral
2. Proliferation of ground substance in supporting tissues of the auditory and vestibular sense organs with loss of fibrous tissue elements, bilateral
3. Loss of dendritic nerve fibers

Title: DFNA9
Chapter: Developmental Defects
Chapter section: Non-Syndromic Hearing Loss / DFNA 9
TB Case Number: 736
Comments: DFNA 9 with COCH mutation
Gender: Female
Age(yrs.): 59
Otologic Dx: 1. Hereditary sensorineural deafness, severe, bilateral
2. Proliferation of ground substance in supporting tissues of the auditory and vestibular sense organs with loss of fibrous tissue elements, bilateral
3. Loss of dendritic nerve fibers