Pontine tegmental cap dysplasia: the neurotologic perspective.
Otolaryngol Head Neck Surg
; 145(6): 992-8, 2011 Dec.
Article
em En
| MEDLINE
| ID: mdl-21705787
ABSTRACT
OBJECTIVES:
Pontine tegmental cap dysplasia (PTCD) is a rare congenital malformation. Clinical and imaging findings in 3 patients and the authors' experience with bilateral cochlear implantation in 1 patient are described. STUDYDESIGN:
Retrospective review.SETTING:
Two tertiary medical centers. SUBJECTS ANDMETHODS:
Three patients were evaluated by an otolaryngologist and underwent magnetic resonance imaging (MRI) of the temporal bones and brain. High-resolution computed tomography (CT) scanning of the temporal bones was performed in 2 patients. Imaging findings of the brain, the presence and course of resolvable cranial nerves, the membranous labyrinth, and internal auditory canals were reviewed. Clinical data were reviewed.RESULTS:
All patients demonstrated typical brain characteristics of PTCD. Mild, bilateral cochlear dysplasia was noted in 2, and all had a normal vestibular labyrinth. The cochleovestibular nerves were universally absent bilaterally. The facial nerves were subjectively deficient bilaterally in 1 patient, unilaterally in the second patient, and normal in the third. An accessory canal for the seventh cranial nerve, referred to as a duplicated internal auditory canal, was present in all patients. Auditory brainstem response testing revealed profound bilateral sensorineural hearing loss in all of the patients; none suffered facial weakness. A single patient underwent bilateral cochlear implantation with only minimal response.CONCLUSION:
The authors report 3 cases of PTCD with emphasis on imaging of the seventh and eighth cranial nerves and clinical neurotologic findings. All patients manifested duplicated internal auditory canals, a previously unreported finding in PTCD. Bilateral profound sensorineural hearing loss is due to absence of the cochleovestibular nerve. Prognosis for cochlear implantation is poor.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Nervo Vestibular
/
Tronco Encefálico
/
Ponte
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Perda Auditiva Neurossensorial
/
Malformações do Sistema Nervoso
Tipo de estudo:
Observational_studies
/
Prognostic_studies
Limite:
Child
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Female
/
Humans
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Infant
/
Male
Idioma:
En
Ano de publicação:
2011
Tipo de documento:
Article