Sensorineural hearing loss in sickle cell disease-A prospective study from Oman

Muhammed Hesham Al Okbi, Salam Alkindi, Rashid K. Al Abri, John Mathew, Afarida A. Nagwa, Anil V. Pathare

Research output: Contribution to journalArticle

10 Citations (Scopus)

Abstract

Background: Sickle cell disease (SCD) is characterized by intermittent episodes of vascular occlusion and end-organ damage. Neurologic symptoms are frequent and auditory involvement is not unexpected. Aim: To study the prevalence and pattern of hearing loss in Omani patients with SCD. Methods: We conducted a prospective case control study on SCD patients attending the outpatient department. Age and sex matched normal volunteer blood donor controls were recruited after an informed consent and Medical Ethics Committee approval. Pure tone audiometry was performed in all cases studied. Results: Forty-six SCD patients (15 males, 32.6%) aged 16-45 years with a mean age of 26 years ± 6.9 and 29 controls (10 males, 34.4%) aged 16-39 years with a mean age of 25.24 ± 8.2 were enrolled in this study. The average hearing thresholds of SCD patients were consistently higher than controls in all frequencies tested in both ears. Of the 92 ears tested in SCD patients, 29.34% had SNHL. Ten patients had bilateral SNHL; whereas 3 and 4 cases had SNHL in left and right ears, respectively. All the control subjects had hearing thresholds within normal limits. Summary/Conclusions: The study reveals a significant incidence of sensorineural hearing loss (SNHL) in SCD patients (36.95%), although the patients were clinically asymptomatic. The hearing loss was worse in the right ears and had a female preponderance. Also, the hearing loss was more severe at the higher frequencies, 2,000-8,000 Hz in SCD patients. HbS, HbF, or low hemoglobin levels did not discriminate SCD patients with SNHL, and the role of hemoglobin F in the cochlea is still not clear. Regular audiometric assessment should therefore be recommended in SCD patients routinely.

Original languageEnglish
Pages (from-to)392-396
Number of pages5
JournalLaryngoscope
Volume121
Issue number2
DOIs
Publication statusPublished - Feb 2011

Fingerprint

Oman
Sensorineural Hearing Loss
Sickle Cell Anemia
Prospective Studies
Ear
Hearing Loss
Hearing
Bilateral Hearing Loss
Pure-Tone Audiometry
Fetal Hemoglobin
Ethics Committees
Medical Ethics
Cochlea
Neurologic Manifestations
Blood Donors
Informed Consent
Blood Vessels
Case-Control Studies
Healthy Volunteers
Hemoglobins

Keywords

  • Level of Evidence: 3A.
  • SCD
  • SNHL

ASJC Scopus subject areas

  • Otorhinolaryngology

Cite this

Sensorineural hearing loss in sickle cell disease-A prospective study from Oman. / Okbi, Muhammed Hesham Al; Alkindi, Salam; Al Abri, Rashid K.; Mathew, John; Nagwa, Afarida A.; Pathare, Anil V.

In: Laryngoscope, Vol. 121, No. 2, 02.2011, p. 392-396.

Research output: Contribution to journalArticle

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abstract = "Background: Sickle cell disease (SCD) is characterized by intermittent episodes of vascular occlusion and end-organ damage. Neurologic symptoms are frequent and auditory involvement is not unexpected. Aim: To study the prevalence and pattern of hearing loss in Omani patients with SCD. Methods: We conducted a prospective case control study on SCD patients attending the outpatient department. Age and sex matched normal volunteer blood donor controls were recruited after an informed consent and Medical Ethics Committee approval. Pure tone audiometry was performed in all cases studied. Results: Forty-six SCD patients (15 males, 32.6{\%}) aged 16-45 years with a mean age of 26 years ± 6.9 and 29 controls (10 males, 34.4{\%}) aged 16-39 years with a mean age of 25.24 ± 8.2 were enrolled in this study. The average hearing thresholds of SCD patients were consistently higher than controls in all frequencies tested in both ears. Of the 92 ears tested in SCD patients, 29.34{\%} had SNHL. Ten patients had bilateral SNHL; whereas 3 and 4 cases had SNHL in left and right ears, respectively. All the control subjects had hearing thresholds within normal limits. Summary/Conclusions: The study reveals a significant incidence of sensorineural hearing loss (SNHL) in SCD patients (36.95{\%}), although the patients were clinically asymptomatic. The hearing loss was worse in the right ears and had a female preponderance. Also, the hearing loss was more severe at the higher frequencies, 2,000-8,000 Hz in SCD patients. HbS, HbF, or low hemoglobin levels did not discriminate SCD patients with SNHL, and the role of hemoglobin F in the cochlea is still not clear. Regular audiometric assessment should therefore be recommended in SCD patients routinely.",
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