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Hearing and otopathology in Crouzon syndrome.
Laryngoscope. 1999 Sep; 109(9):1372-5.L

Abstract

OBJECTIVES

To better establish the incidence and types of otologic and auditory abnormalities in patients with Crouzon syndrome.

STUDY DESIGN

Retrospective chart review of the otologic and auditory findings of patients diagnosed with Crouzon syndrome who were seen at our institution between 1978 and 1994.

METHODS

Charts were reviewed and data recorded on patient sex, family history, appearance, auricular abnormalities, auditory findings, history of otologic disease, and follow-up.

RESULTS

Nineteen patients were identified with the diagnosis of Crouzon syndrome: 12 males and 7 females. Twelve cases represented spontaneous mutations. Eight patients had abnormalities involving the external ear: from malalignment of the pinna (6 patients) to external auditory canal atresia (1 patient). Ten patients had documented hearing loss: 4 with conductive hearing loss, 2 with a mixed hearing loss, and 4 with a sensorineural hearing loss, the etiologies of which ranged from ossicular fixation and serous otitis media to unknown sensorineural deficits.

CONCLUSIONS

Patients with Crouzon syndrome can exhibit various pathological features of the ear. Although external malformations are unusual, middle ear disease and hearing loss are common. We advocate close otologic and audiologic follow-up in these patients and note a higher frequency of sensorineural hearing loss than previously reported. Recent genetic advances may allow more accurate and earlier diagnosis of this syndrome.

Authors+Show Affiliations

Department of Otorhinolaryngology, Mayo Clinic, Rochester, Minnesota 55905, USA.No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

10499038

Citation

Orvidas, L J., et al. "Hearing and Otopathology in Crouzon Syndrome." The Laryngoscope, vol. 109, no. 9, 1999, pp. 1372-5.
Orvidas LJ, Fabry LB, Diacova S, et al. Hearing and otopathology in Crouzon syndrome. Laryngoscope. 1999;109(9):1372-5.
Orvidas, L. J., Fabry, L. B., Diacova, S., & McDonald, T. J. (1999). Hearing and otopathology in Crouzon syndrome. The Laryngoscope, 109(9), 1372-5.
Orvidas LJ, et al. Hearing and Otopathology in Crouzon Syndrome. Laryngoscope. 1999;109(9):1372-5. PubMed PMID: 10499038.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Hearing and otopathology in Crouzon syndrome. AU - Orvidas,L J, AU - Fabry,L B, AU - Diacova,S, AU - McDonald,T J, PY - 1999/9/28/pubmed PY - 1999/9/28/medline PY - 1999/9/28/entrez SP - 1372 EP - 5 JF - The Laryngoscope JO - Laryngoscope VL - 109 IS - 9 N2 - OBJECTIVES: To better establish the incidence and types of otologic and auditory abnormalities in patients with Crouzon syndrome. STUDY DESIGN: Retrospective chart review of the otologic and auditory findings of patients diagnosed with Crouzon syndrome who were seen at our institution between 1978 and 1994. METHODS: Charts were reviewed and data recorded on patient sex, family history, appearance, auricular abnormalities, auditory findings, history of otologic disease, and follow-up. RESULTS: Nineteen patients were identified with the diagnosis of Crouzon syndrome: 12 males and 7 females. Twelve cases represented spontaneous mutations. Eight patients had abnormalities involving the external ear: from malalignment of the pinna (6 patients) to external auditory canal atresia (1 patient). Ten patients had documented hearing loss: 4 with conductive hearing loss, 2 with a mixed hearing loss, and 4 with a sensorineural hearing loss, the etiologies of which ranged from ossicular fixation and serous otitis media to unknown sensorineural deficits. CONCLUSIONS: Patients with Crouzon syndrome can exhibit various pathological features of the ear. Although external malformations are unusual, middle ear disease and hearing loss are common. We advocate close otologic and audiologic follow-up in these patients and note a higher frequency of sensorineural hearing loss than previously reported. Recent genetic advances may allow more accurate and earlier diagnosis of this syndrome. SN - 0023-852X UR - https://www.unboundmedicine.com/medline/citation/10499038/Hearing_and_otopathology_in_Crouzon_syndrome_ L2 - https://doi.org/10.1097/00005537-199909000-00002 DB - PRIME DP - Unbound Medicine ER -