Audiologic and Otologic Characteristics of Pfeiffer Syndrome
| Autor: | Linda D. Vallino-Napoli |
|---|---|
| Rok vydání: | 1996 |
| Předmět: |
Male
medicine.medical_specialty Hearing loss Ear Middle Otitis Media Suppurative 03 medical and health sciences 0302 clinical medicine Temporal bone otorhinolaryngologic diseases Medicine Humans Prospective Studies Ear External 030223 otorhinolaryngology Child Hearing Loss Retrospective Studies Ossicles medicine.diagnostic_test business.industry Acrocephalosyndactylia medicine.disease Hypoplasia Conductive hearing loss Surgery medicine.anatomical_structure Acoustic Impedance Tests Otorhinolaryngology 030220 oncology & carcinogenesis Child Preschool Middle ear Pfeiffer syndrome Audiometry Pure-Tone Female medicine.symptom Audiometry Oral Surgery business Tomography X-Ray Computed |
| Zdroj: | The Cleft Palate-Craniofacial Journal. 33:524-529 |
| ISSN: | 1545-1569 1055-6656 |
| DOI: | 10.1597/1545-1569(1996)033<0524:aaocop>2.3.co;2 |
| Popis: | Objective To examine the prevalence and type of hearing loss and otopathology in patients with Pfeiffer syndrome. Design Retrospective and prospective study design. Setting A pediatric tertiary care hospital. Subjects Nine patients-ranging in age from 2 to 12 years. Method Hearing levels and middle ear function were assessed using standard procedures. Otoscopy was also conducted. Computerized tomography (CT) scans of the temporal bone were obtained to study outer, middle, and inner ear anatomy. Results Hearing loss was present in eight of the nine patients. The degree of loss varied but was moderate to severe in most patients. Seven patients had conductive hearing loss and one had mixed loss; none had purely sensorineural loss. Four patients had a history of middle ear effusion. Primary CT findings showed stenosis and/or atresia of the external auditory canal, hypoplasia of the middle ear cavity, and an enlarged middle ear cavity. The ossicles were hypoplastic in a few cases. With one exception, inner ear anatomy was normal. Conclusion Otologic malformations and hearing loss are features of Pfeiffer syndrome. Major factors contributing to hearing loss were anatomic abnormalities of the external auditory canal and middle ear, which can be identified by computerized tomography. Otitis media was also present and may have caused or contributed to the hearing loss. We recommend that the computerized tomographic study, which is often used to evaluate and plan treatment for the craniofacial skeleton, be extended to include a thorough evaluation of otologic structures In patients with Pfeiffer syndrome. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|