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Otologic and audiologic features of Nager acrofacial dysostosis.
Int J Pediatr Otorhinolaryngol. 2005 Aug; 69(8):1053-9.IJ

Abstract

OBJECTIVE

To describe the otologic and audiologic characteristics of pediatric patients with Nager acrofacial dysostosis.

DESIGN

Retrospective case series.

SETTING

Multidisciplinary clinic in a tertiary care children's hospital.

SUBJECTS

Patients less than 18 years of age with Nager acrofacial dysostosis.

METHODS

Nager syndrome is a mandibulofacial dysostosis associated with preaxial limb abnormalities and multiple craniofacial anomalies. Ten patients with Nager syndrome were reviewed. Relevant literature, 1966 to the present, was reviewed with the assistance of Medline.

RESULTS

External and middle ear abnormalities are common in Nager syndrome. All non-atretic ears had significant difficulty with otitis media, requiring an average of two sets of tympanostomy tubes. Cholesteatoma was diagnosed in one patient. Pure conductive hearing loss was identified in eight patients with mixed hearing loss noted in two patients. Conductive hearing loss greater than 30 dB HL was noted in 90% (9/10) of patients, with 40% (4/10) having 55-70 dB HL loss. Although amplification was effective, results of surgical interventions to correct conductive hearing loss were inconsistent. Two patients with mixed hearing loss developed the sensorineural component in later childhood, indicating that progressive or fluctuating sensorineural hearing loss is also possible in this population.

CONCLUSIONS

Pediatric patients with Nager acrofacial dysostosis exhibit conductive hearing loss due to middle and external ear pathology. Prolonged ventilation of the middle ear via tympanostomy tubes and amplification with hearing aids are often required. Some patients also demonstrate mixed hearing loss that may be progressive and should be monitored carefully. Early and aggressive management in a multidisciplinary team approach is recommended.

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Publisher Full Text

Authors+Show Affiliations

Herrmann BW
Washington University School of Medicine, Department of Otolaryngology-Head and Neck Surgery, Washington, DC, USA. bhermann@childrensent.com
Karzon R
No affiliation info available
Molter DW
No affiliation info available

MeSH

Audiometry, Pure-ToneChild, PreschoolCholesteatomaCraniofacial DysostosisEar, ExternalEar, MiddleFemaleFollow-Up StudiesHearing Loss, ConductiveHumansInfantLimb Deformities, CongenitalMaleMastoidOtitis MediaRetrospective StudiesSyndromeTomography, X-Ray Computed

Pub Type(s)

Journal Article

Language

eng

PubMed ID

16005346

Citation

Herrmann, Brian W., et al. "Otologic and Audiologic Features of Nager Acrofacial Dysostosis." International Journal of Pediatric Otorhinolaryngology, vol. 69, no. 8, 2005, pp. 1053-9.
Herrmann BW, Karzon R, Molter DW. Otologic and audiologic features of Nager acrofacial dysostosis. Int J Pediatr Otorhinolaryngol. 2005;69(8):1053-9.
Herrmann, B. W., Karzon, R., & Molter, D. W. (2005). Otologic and audiologic features of Nager acrofacial dysostosis. International Journal of Pediatric Otorhinolaryngology, 69(8), 1053-9.
Herrmann BW, Karzon R, Molter DW. Otologic and Audiologic Features of Nager Acrofacial Dysostosis. Int J Pediatr Otorhinolaryngol. 2005;69(8):1053-9. PubMed PMID: 16005346.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Otologic and audiologic features of Nager acrofacial dysostosis. AU - Herrmann,Brian W, AU - Karzon,Roanne, AU - Molter,David W, Y1 - 2005/03/19/ PY - 2004/10/11/received PY - 2005/02/09/revised PY - 2005/02/09/accepted PY - 2005/7/12/pubmed PY - 2006/5/26/medline PY - 2005/7/12/entrez SP - 1053 EP - 9 JF - International journal of pediatric otorhinolaryngology JO - Int J Pediatr Otorhinolaryngol VL - 69 IS - 8 N2 - OBJECTIVE: To describe the otologic and audiologic characteristics of pediatric patients with Nager acrofacial dysostosis. DESIGN: Retrospective case series. SETTING: Multidisciplinary clinic in a tertiary care children's hospital. SUBJECTS: Patients less than 18 years of age with Nager acrofacial dysostosis. METHODS: Nager syndrome is a mandibulofacial dysostosis associated with preaxial limb abnormalities and multiple craniofacial anomalies. Ten patients with Nager syndrome were reviewed. Relevant literature, 1966 to the present, was reviewed with the assistance of Medline. RESULTS: External and middle ear abnormalities are common in Nager syndrome. All non-atretic ears had significant difficulty with otitis media, requiring an average of two sets of tympanostomy tubes. Cholesteatoma was diagnosed in one patient. Pure conductive hearing loss was identified in eight patients with mixed hearing loss noted in two patients. Conductive hearing loss greater than 30 dB HL was noted in 90% (9/10) of patients, with 40% (4/10) having 55-70 dB HL loss. Although amplification was effective, results of surgical interventions to correct conductive hearing loss were inconsistent. Two patients with mixed hearing loss developed the sensorineural component in later childhood, indicating that progressive or fluctuating sensorineural hearing loss is also possible in this population. CONCLUSIONS: Pediatric patients with Nager acrofacial dysostosis exhibit conductive hearing loss due to middle and external ear pathology. Prolonged ventilation of the middle ear via tympanostomy tubes and amplification with hearing aids are often required. Some patients also demonstrate mixed hearing loss that may be progressive and should be monitored carefully. Early and aggressive management in a multidisciplinary team approach is recommended. SN - 0165-5876 UR - https://www.unboundmedicine.com/medline/citation/16005346/Otologic_and_audiologic_features_of_Nager_acrofacial_dysostosis_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0165-5876(05)00101-1 DB - PRIME DP - Unbound Medicine ER -