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Otologic manifestations of malignant osteopetrosis.
Otol Neurotol. 2005 Jul; 26(4):762-6.ON

Abstract

OBJECTIVE

To determine the incidence of hearing loss and describe the neurotologic manifestations over time in a large series of patients with malignant osteopetrosis.

STUDY DESIGN

Longitudinal study.

SETTING

Tertiary care referral center.

PATIENTS

Thirty-two patients, including 19 infants (< 1 yr of age at initial visit) and 13 children (aged 1-7.6 yr at initial visit), with autosomal recessive osteopetrosis were followed-up during a 10-year period from 1991 to 2001. The average length of follow-up was 2.5 years (range, 0-9.1 yr).

INTERVENTIONS

Patients underwent annual otologic evaluations including clinical examination, audiologic evaluation (auditory brainstem response, pure-tone thresholds, speech discrimination scores, and tympanograms), and high-resolution computed tomography of the temporal bone.

MAIN OUTCOME MEASURES

Incidence of hearing loss, otitis media, and facial paralysis. Serial changes in temporal bone anatomy by computed tomographic scan.

RESULTS

Twenty-six percent of infants' ears demonstrated hearing loss during the first year of life, and 78% of children's ears demonstrated hearing loss during the study period. Of the children's ears with hearing loss, 100% had a conductive component and 26% had an additional sensorineural component (mixed hearing loss); VIIIth nerve conduction was normal in 100% of infants and 78% of children. Five patients (16%) had unilateral facial nerve palsy. Common temporal bone findings included thickening and sclerosis of the calvarium; poor pneumatization of the mastoid bone; and narrowing of the external auditory canal, eustachian tube, and internal auditory canal.

CONCLUSION

Otologic manifestations are common in malignant osteopetrosis secondary to the formation of dense, brittle bone. Frequent findings include external auditory canal stenosis, otitis media, conductive and sensorineural hearing loss, and facial nerve paralysis.

Authors+Show Affiliations

Department of Otolaryngology-Head and Neck Surgery, Medical University of South Carolina, Charleston, South Carolina 29425, USA. dozierts@musc.eduNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

16015181

Citation

Dozier, Thomas S., et al. "Otologic Manifestations of Malignant Osteopetrosis." Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology, vol. 26, no. 4, 2005, pp. 762-6.
Dozier TS, Duncan IM, Klein AJ, et al. Otologic manifestations of malignant osteopetrosis. Otol Neurotol. 2005;26(4):762-6.
Dozier, T. S., Duncan, I. M., Klein, A. J., Lambert, P. R., & Key, L. L. (2005). Otologic manifestations of malignant osteopetrosis. Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology, 26(4), 762-6.
Dozier TS, et al. Otologic Manifestations of Malignant Osteopetrosis. Otol Neurotol. 2005;26(4):762-6. PubMed PMID: 16015181.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Otologic manifestations of malignant osteopetrosis. AU - Dozier,Thomas S, AU - Duncan,Irenee M, AU - Klein,Alan J, AU - Lambert,Paul R, AU - Key,L Lyndon,Jr PY - 2005/7/15/pubmed PY - 2005/8/18/medline PY - 2005/7/15/entrez SP - 762 EP - 6 JF - Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology JO - Otol Neurotol VL - 26 IS - 4 N2 - OBJECTIVE: To determine the incidence of hearing loss and describe the neurotologic manifestations over time in a large series of patients with malignant osteopetrosis. STUDY DESIGN: Longitudinal study. SETTING: Tertiary care referral center. PATIENTS: Thirty-two patients, including 19 infants (< 1 yr of age at initial visit) and 13 children (aged 1-7.6 yr at initial visit), with autosomal recessive osteopetrosis were followed-up during a 10-year period from 1991 to 2001. The average length of follow-up was 2.5 years (range, 0-9.1 yr). INTERVENTIONS: Patients underwent annual otologic evaluations including clinical examination, audiologic evaluation (auditory brainstem response, pure-tone thresholds, speech discrimination scores, and tympanograms), and high-resolution computed tomography of the temporal bone. MAIN OUTCOME MEASURES: Incidence of hearing loss, otitis media, and facial paralysis. Serial changes in temporal bone anatomy by computed tomographic scan. RESULTS: Twenty-six percent of infants' ears demonstrated hearing loss during the first year of life, and 78% of children's ears demonstrated hearing loss during the study period. Of the children's ears with hearing loss, 100% had a conductive component and 26% had an additional sensorineural component (mixed hearing loss); VIIIth nerve conduction was normal in 100% of infants and 78% of children. Five patients (16%) had unilateral facial nerve palsy. Common temporal bone findings included thickening and sclerosis of the calvarium; poor pneumatization of the mastoid bone; and narrowing of the external auditory canal, eustachian tube, and internal auditory canal. CONCLUSION: Otologic manifestations are common in malignant osteopetrosis secondary to the formation of dense, brittle bone. Frequent findings include external auditory canal stenosis, otitis media, conductive and sensorineural hearing loss, and facial nerve paralysis. SN - 1531-7129 UR - https://www.unboundmedicine.com/medline/citation/16015181/Otologic_manifestations_of_malignant_osteopetrosis_ L2 - https://doi.org/10.1097/01.mao.0000178139.27472.8d DB - PRIME DP - Unbound Medicine ER -