Developmental Defects

Temporal Bone Image Library

  • 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

  • Title: L209 DFNA9
  • Chapter: Developmental Defects
  • Chapter section: Non-Syndromic Hearing Loss / DFNA 9
  • TB Case Number: 399
  • 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: L181 DFNA9
  • Chapter: Developmental Defects
  • Chapter section: Non-Syndromic Hearing Loss / DFNA 9
  • TB Case Number: 399
  • 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: L301 DFNA9
  • Chapter: Developmental Defects
  • Chapter section: Non-Syndromic Hearing Loss / DFNA 9
  • TB Case Number: 399
  • 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: R81 DFNA9
  • Chapter: Developmental Defects
  • Chapter section: Non-Syndromic Hearing Loss / DFNA 9
  • TB Case Number: 399
  • 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, L-209
  • Chapter: Developmental Defects
  • Chapter section: Non-Syndromic Hearing Loss / DFNA 9
  • TB Case Number: 399
  • Comments: DFNA9 with COCH mutation, Movat's pentachrome stain
  • 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, L-209
  • Chapter: Developmental Defects
  • Chapter section: Non-Syndromic Hearing Loss / DFNA 9
  • TB Case Number: 399
  • Comments: DFNA9 with COCH mutation, Movat's pentachrome stain
  • 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, L-209
  • Chapter: Developmental Defects
  • Chapter section: Non-Syndromic Hearing Loss / DFNA 9
  • TB Case Number: 399
  • Comments: DFNA9 with COCH mutation, Movat's pentachrome stain, saccule
  • 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, L-209
  • Chapter: Developmental Defects
  • Chapter section: Non-Syndromic Hearing Loss / DFNA 9
  • TB Case Number: 399
  • Comments: DFNA9 with COCH mutation, Movat's pentachrome stain, Posterior 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: L-247 (unaffected)
  • Chapter: Developmental Defects
  • Chapter section: Non-Syndromic Hearing Loss / DFNA 9
  • TB Case Number: 733
  • Comments: Movat's pentachrome stain
  • Gender: Male
  • Age(yrs.): 81
  • otologic_dx:

    1. Sensorineural hearing loss due to aging, combination strial atrophy and inner ear conductive lesion

    2. Acoustic trauma, bilateral, mild

    3. Excellent anatomical preparation

  • Title: L-247 (unaffected)
  • Chapter: Developmental Defects
  • Chapter section: Non-Syndromic Hearing Loss / DFNA 9
  • TB Case Number: 733
  • Comments: Movat's pentachrome stain
  • Gender: Male
  • Age(yrs.): 81
  • otologic_dx:

    1. Sensorineural hearing loss due to aging, combination strial atrophy and inner ear conductive lesion

    2. Acoustic trauma, bilateral, mild

    3. Excellent anatomical preparation

  • Title: L-247 (unaffected)
  • Chapter: Developmental Defects
  • Chapter section: Non-Syndromic Hearing Loss / DFNA 9
  • TB Case Number: 733
  • Comments: Movat's pentachrome stain, saccule
  • Gender: Male
  • Age(yrs.): 81
  • otologic_dx:

    1. Sensorineural hearing loss due to aging, combination strial atrophy and inner ear conductive lesion

    2. Acoustic trauma, bilateral, mild

    3. Excellent anatomical preparation

  • Title: R-303 (unaffected)
  • Chapter: Developmental Defects
  • Chapter section: Non-Syndromic Hearing Loss / DFNA 9
  • TB Case Number: 733
  • Comments: Movat's pentachrome stain, Posterior ampulla
  • Gender: Male
  • Age(yrs.): 81
  • otologic_dx:

    1. Sensorineural hearing loss due to aging, combination strial atrophy and inner ear conductive lesion

    2. Acoustic trauma, bilateral, mild

    3. Excellent anatomical preparation

  • Title: DFNA9, R-580
  • Chapter: Developmental Defects
  • Chapter section: Non-Syndromic Hearing Loss / DFNA 9
  • Comments: from House Ear Institute- DFNA9
  • Title: DFNA9 case from House Ear Institute
  • Chapter: Developmental Defects
  • Chapter section: Non-Syndromic Hearing Loss / DFNA 9
  • Comments: There is an abnormal deposition of an acellular eosinophilic material in the incudo-malleal joint
  • Title: DFNA9 case from House Ear Institute
  • Chapter: Developmental Defects
  • Chapter section: Non-Syndromic Hearing Loss / DFNA 9
  • Comments: There is an abnormal deposition of an acellular eosinophilic material in the incudo-malleal joint
  • Title: Waardenburg type I, R-91
  • Chapter: Developmental Defects
  • Chapter section: Syndromic Hearing Loss / Waardenburg Syndrome
  • TB Case Number: 907
  • Comments: Waardenburg type I
  • Gender: Female
  • Age(yrs.): 76
  • otologic_dx:

    1. Waardenburg’s syndrome, type I, with sensorineural hearing loss, right
    2. Dysmorphogenesis, cochlea and vestibular labyrinth, due to Waardenburg’s syndrome, right
    3. Jugular bulb, high, left

  • Title: Waardenburg type I, L-81
  • Chapter: Developmental Defects
  • Chapter section: Syndromic Hearing Loss / Waardenburg Syndrome
  • TB Case Number: 907
  • Comments: Waardenburg type I
  • Gender: Female
  • Age(yrs.): 76
  • otologic_dx:

    1. Waardenburg’s syndrome, type I, with sensorineural hearing loss, right

    2. Dysmorphogenesis, cochlea and vestibular labyrinth, due to Waardenburg’s syndrome, right

    3. Jugular bulb, high, left

  • Title: Waardenburg type I, R-181
  • Chapter: Developmental Defects
  • Chapter section: Syndromic Hearing Loss / Waardenburg Syndrome
  • TB Case Number: 907
  • Comments: Waardenburg type I
  • Gender: Female
  • Age(yrs.): 76
  • otologic_dx:

    1. Waardenburg’s syndrome, type I, with sensorineural hearing loss, right

    2. Dysmorphogenesis, cochlea and vestibular labyrinth, due to Waardenburg’s syndrome, right

    3. Jugular bulb, high, left

  • Title: Waardenburg type I, L-201
  • Chapter: Developmental Defects
  • Chapter section: Syndromic Hearing Loss / Waardenburg Syndrome
  • TB Case Number: 907
  • Comments: Waardenburg type I
  • Gender: Female
  • Age(yrs.): 76
  • otologic_dx:

    1. Waardenburg’s syndrome, type I, with sensorineural hearing loss, right

    2. Dysmorphogenesis, cochlea and vestibular labyrinth, due to Waardenburg’s syndrome, right

    3. Jugular bulb, high, left

  • Title: Waardenburg type I, R-91
  • Chapter: Developmental Defects
  • Chapter section: Syndromic Hearing Loss / Waardenburg Syndrome
  • TB Case Number: 907
  • Comments: Waardenburg type I
  • Gender: Female
  • Age(yrs.): 76
  • otologic_dx:

    1. Waardenburg’s syndrome, type I, with sensorineural hearing loss, right

    2. Dysmorphogenesis, cochlea and vestibular labyrinth, due to Waardenburg’s syndrome, right

    3. Jugular bulb, high, left