Abnormal Auditory Brainstem Response (ABR) Findings in a Near-Normal Hearing Child with Noonan Syndrome

Document Type : Case Report

Authors

1 Department of Audiology, Faculty of Rehabilitation, Iran University of Medical Sciences, Tehran, Iran.

2 Audiology Program, School of Health Sciences, University Sains Malaysia, Kelantan, Malaysia.

3 Department of Otorhinolaryngology, School of Medical Sciences, University Sains Malaysia, Kelantan, Malaysia.

Abstract

Introduction:
Noonan syndrome (NS) is a heterogeneous genetic disease that affects many parts of the body. It was named after Dr. Jacqueline Anne Noonan, a paediatric cardiologist.
Case Report:
We report audiological tests and auditory brainstem response (ABR) findings in a 5-year old Malay boy with NS. Despite showing the marked signs of NS, the child could only produce a few meaningful words. Audiological tests found him to have bilateral mild conductive hearing loss at low frequencies. In ABR testing, despite having good waveform morphology, the results were atypical. Absolute latency of wave V was normal but interpeak latencies of wave’s I-V, I-II, II-III were prolonged. Interestingly, interpeak latency of waves III-V was abnormally shorter.
Conclusion:
Abnormal ABR results are possibly due to abnormal anatomical condition of brainstem and might contribute to speech delay.

Keywords

Main Subjects

References

1. Romano AA, AllansonJE, Dahlgren J, Gelb BD, Hall B, Pierpont ME, et al., Noonan Syndrome: Clinical Features, Diagnosis and Management Guidelines. Paediatrics.2010;126(4): 746.
2. Burgt IVD, Noonan Syndrome. Orphanet Journal of Rare Diseases.2007; 2:4.
3. SharlandM, Burch M, Mckenna WM, Patton MA. A clinical study of Noonan Syndrome. Arch Dis Child.1992; 67:178-83.
4. Mendez HMM, Optiz JM. Noonan Syndrome: a review. Am J Med Genet. 1985; 21(3): 493- 506.
5.  Nora JJ, Nora AH, Sinha AK,Spangler R, Lubs H. The Ullrich-Noonan syndrome (Turner phenotype). Am J Dis Child.1974;127(1):48-55.
6. Heller RH. The Turner phenotype in the male. The journal of Pediatrics.1965; 66(1): 48-63. 
7. Duenas DA, Preissig S,Summitt RL,Wilroy RS,Lemmi H,Dews JE. Neurologic manifestations of the Noonan syndrome. South Medical Journal. 1973; 66(2):193-6.
8. Cremers CW, Van der Burgt CJ. Hearing loss in Noonan syndrome.Int J PediatrOtorhinolaryngol. 1992; 23(1): 81- 4.
9. Miura M, Sando I, Orita Y, Hirsch BE. Temporal bone histopathological study of Noonan syndrome. IntJournal of Pediatric Otorhinolaryngo- logy. 2001;60: 73-82.
10. Van Trier DC, Van Nierop J, Draaisma JM, Burgt IV, Kunst H, Croonen EA et al. External ear anomalies and hearing impairment in Noonan Syndrome. Int J PediatrOtorhinolaryngol. 2015; 79(6): 874-8.
11. Takahara T, Sando I. Mesenchyme remaining in temporal bones from patients with congenital anomalies. A quantitative histopathologic study. Ann. Otol. Rhinolaryngol. 1987;96(3): 333–9.
12. Matas CG, Leite RA, Goncalves IC, Neves IF. Brainstem Auditory Evoked Potential in Individuals with Conductive and Sensorineural Hearing Losses. 2005; 9(4): 337.
13. Hall J. New handbook of auditory evoked responses. Boston Mass. Pearson. 2007; 232:3.
14. Sand T, The choice of ABR click polarity and amplitude variables in multiple sclerosis patients. ScandAudiol. 1991;20(1) 75-80.
15. Kao CP. Evaluation and management of the child with speech delay. Am Fam Physician.1999; 59(11): 3121-8.
Iranian Journal of Otorhinolaryngology
Volume 29, Issue 1
January and February 2017
Pages 53-57

Files

Share

How to cite

Statistics