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• W2186093357 abstract "Original ArticlesSensorineural Hearing Loss in Homozygous Sickle Cell Disease in Qatif, Saudi Arabia Ibrahim A. Al-Dabbous, MBBS, DCH, CABP Ali H. Al Jam'a, MBBS, CABIM Suresh K. Obeja, MBBS, MS, DLO A. N. Raj Murugan, and MB, MS, FRCS, DLO Haroun A. HammadMBBS, DLO, FRCS Ibrahim A. Al-Dabbous Address reprint requests and correspondence to Dr. Al-Dabbous: P.O. Box 628, Qatif 31911, Eastern Province, Saudi Arabia. From the Department of Pediatrics (Dr. Al-Dabbous), Qatif Central Hospital, Qatif Search for more papers by this author , Ali H. Al Jam'a From the Department of Internal Medicine, Qatif Central Hospital, Qatif Search for more papers by this author , Suresh K. Obeja From the Department of ENT, Qatif Central Hospital, Qatif Search for more papers by this author , A. N. Raj Murugan From the Department of ENT, Qatif Central Hospital, Qatif Search for more papers by this author , and Haroun A. Hammad From the Department of ENT, Qatif Central Hospital, Qatif Search for more papers by this author Published Online:1 Nov 1996https://doi.org/10.5144/0256-4947.1996.641SectionsPDF ToolsAdd to favoritesDownload citationTrack citations ShareShare onFacebookTwitterLinked InRedditEmail AboutAbstractSensorineural hearing loss (SNHL) is a known complication of sickle cell disease (SCD). There is a paucity of information on this problem in Saudi SCD patients. A prospective controlled study was conducted over 27 months in Qatif Central Hospital. One hundred patients with sickle cell anemia (SCA), aged five to 40 years, were studied. The control group consisted of 100 healthy individuals. A detailed history, otologic and audiologic examinations were carried out on both groups. Further investigations to rule out local retrocochlear pathology were performed on those having unilateral SNHL. No case of SNHL was detected in the control group, while it was recorded in 19 (19%) of SCA patients, 13 males and six females. There was a significant association between the SNHL and the onset of first vaso-occlusive crisis at six years of age or less. There was no significant difference in the frequency of SNHL between children and adults. Twenty-one percent of patients who had SNHL completely recovered. Patients with SCD need a careful regular hearing assessment. Those detected to have SNHL need a careful plan implemented by both the clinician and otolaryngologist for proper care of this important complication.IntroductionSensorineural hearing loss (SNHL) has been recognized as a complication in patients with sickle cell disease (SCD) for over 20 years.1 It was reported that patients with SCD have a much higher incidence of this complication than the rest of the population,2,3 with a variable degree of severity.2,4–6 There is very limited information about this complication in the so-called “benign” Saudi SCD.7This study was conducted to determine the frequency and the pattern of SNHL among homozygous sickle cell anemia (SCA) patients in Qatif and to look for possible etiologic factors.To the best of our knowledge, this is the largest series of homozygous SCD patients studied for SNHL.MATERIAL AND METHODSA prospective study was conducted at Qatif Central Hospital (QCH) from April 1992 through July 1994. All patients with SCA aged five years to 40 years and attending pediatric and adult sickle cell clinics steadily were included.The diagnosis of SCA was made on the basis of clinical as well as positive sickling test and hemoglobin electrophoresis, using Helena Laboratories Super Z Electrophoresis Kit.8The control group was matched for age and sex. They were documented to have normal hemoglobin electrophoresis with AA pattern, with no other hematologic disease.The age limit was necessary, as young patients (<five years) are generally inconsistent in their performance on hearing tests and older patients (>40 years) might suffer the effect of presbycusis.A detailed history of the clinical course of the disease was obtained by reviewing each patient's case file and interviewing the patients or parents of young patients. The age at first attack was recorded. Patients with a history of meningitis, mumps (or parotid swelling), head trauma, noise exposure, ear discharge, intake of ototoxic drugs, Meniere's disease or family history of hearing loss were excluded.Otologic and audiologic examinations were conducted on all patients by one experienced ENT specialist. Their ears were examined and the appearance of the tympanic membrane was noted, after taking out any cerumen present. Simple tuning fork tests (Rinne and Weber) were carried out in order to identify possible conduction hearing loss (CHL). Audiological evaluation consisted of tympanogram, acoustic reflex threshold (ART) measurement, and pure tone audiometry. VDRL was done on all patients with hearing loss, whereas ART, CT scan of petrous bone and brain, and brainstem-evoked response audiometry (BERA) were done only on those patients having unilateral SNHL. Madsen ZO-2020 emittance system was used to obtain tympanometry and ART data. Medelec Sapphire 2A was used to obtain BERA. Jerger's differentiation of tympanogram into type A, B and C was employed to analyze the study data.9 Contralateral ART for a subject was considered abnormal or elevated if ART was 110 dB or greater at two or more frequencies from 500 through 2000 Hz. The pure tone audiometer used in this study was calibrated to the American National Standard Institute, 1969 Standard.10 Hearing levels were assessed separately in each ear in a soundproof booth at frequencies of 500, 1000, 2000, 4000 and 8000 Hz. Those who had hearing losses involving frequencies above 2000 Hz were classified as high-frequency losses.11 Measurements were made on a model A-17 Ambco Audiometer calibrated to International Standard and expressed as decibels (dB). Pure tone threshold of 25 dB or more in one or more test frequencies from 500 through 8000 Hz was considered a hearing loss for the purpose of the present investigation. Follow-up audiological assessment was done for a period of six months to two years.Hematologic parameters (hemoglobin, MCV, HbF and G6PD status) in patients with and without SNHL were compared.Chi-squared test and Mann-Whitney test for independent observations were used to compare the data for patients with and without SNHL.RESULTSOtologic and audiologic examinations were conducted on 100 SCA patients and 100 healthy individuals (control). The SCA patients were made up of 41 children under 13 years (23 males and 18 females) and 59 adults >13 years (35 males and 24 females). In the control group, there were 43 children <13 years (23 males and 20 females) and 57 adults ≥13 years (36 males and 21 females). There was no significant difference in the distribution of age or sex over the SCA patients and the control group (P values were 0.77 and 0.88 respectively).Except for slight differences in compliance, tympanometry was normal in both the SCA and control group. Sensorineural hearing loss of more than 25 dB at two or more frequencies was recorded in 19 of the 100 SCA patients (19%) and in none of the control individuals. There were five pediatric patients (≤12 years) and 14 adults (>12 years) (Table 1). The difference was not statistically significant (x2=1.4, P=0.24). The youngest patient with SNHL was six years old and the oldest was 40 years of age. Thirteen patients with SNHL were male and six patients were female (Table 2). The difference was also not statistically significant (x2=0.58, P=0.44). Most of the patients had a moderate degree of SNHL (Table 3), and three patients had high-frequency losses. Bilateral SNHL was found in nine patients (47.4%). The 10 patients with unilateral hearing loss were investigated to rule out retrocochlear pathology. The results of ART, computerized tomography of the internal auditory canals and brain and BERA were normal. VDRL was nonreactive in all patients with SNHL. A history of first attack was obtained from 69 SCA patients. Eighteen patients (out of 19) with SNHL (94.7%) had their first attack at ≤six years of age, in contrast to 37 patients (out of 50) without SNHL (74%) (P=0.004). No patient with SNHL had suffered from CNS infarction in the past.Table 1. Age distribution of SCA with and without SNHL.Table 2. Sex distribution of SCA with and without SNHL.Table 3. Degree of sensorineural hearing loss.Comparison of hematologic parameters in patients with and without SNHL revealed that MCV and HbF were lower in patients with SNHL with statistically significant difference (Table 4). G6PD status was studied in 70 SCA. Out of these, 27 patients were G6PD deficient. Among patients with G6PD deficiency, 10 patients had SNHL, with no statistically significant difference (P=0.23).Table 4. Hematologic indices in patients with and without SNHLOut of 19 patients with SNHL, four patients (21.1%) regained spontaneous normal hearing over 18-21 months. Fifteen to 40 dB hearing improvement was found in seven patients (36.8%) over six to 21 months. No specific treatment was given to these patients.DISCUSSIONSickle cell anemia is a chronic hemolytic disorder, manifested by a wide variety of clinical and hematological features and complications. One of these complications is sensorineural hearing loss. There is a consensus in the various audiological studies that patients with SCA have a much higher incidence of SNHL than the rest of the population.12 The frequency of this complication was variable, ranging from 12% to 29%.2,3,6,7,12,13 Our results of 19% are similar to the previous reports from Saudi Arabia (23.8%),7 as well as from Nigeria (21.4%)3 and Jamaica (21.7%).6 Studies from developed countries revealed much lower frequency of this complication (12% in the US2 and 13.5% in the UK12). This finding may suggest that better care may decrease the incidence of SNHL.In this study, we did not find an increase in the frequency of hearing loss with increasing age, a finding which was supported by Friedman et al.2 and Ajulo et al.12 This is in disagreement with others who did find an increased incidence of hearing loss with increasing age.6The relationship between SNHL and early occurrence of attacks was suggested by Odetoyinbo and Adekile, who found that more than 90% of those who developed SNHL had their first vaso-occlusive crisis before the age of five years.3 Our study proved such a relationship. This may indicate that early exposure of the cochlea to vasoocclusive episodes during sickle cell crisis may cause cochlear damage, leading to SNHL. Koide et al. and Serjeant et al. have suggested that vaso-occlusion of the cochlear blood vessels (a cochlear origin) is the cause of the hearing loss.14,15A possible neural cause of SNHL in SCA was suggested by various authors.2–4 In our study, retrocochlear pathology was excluded in patients with unilateral SNHL by acoustic reflex threshold, CT scan of petrous bone and brainstem-evoked response audiometry. This was supported by other studies.12 No patient with SNHL in our study had suffered from CNS infarction in the past. These findings were suggestive of nonneural causes of SNHL in our patients.High HbF,16,17 alpha-thalassemia17,18 and G6PD deficiency19,20 have been considered as ameliorating factors in SCD, although disputed by others.19,21 In this study, lower HbF among those with SNHL with statistically significant difference may suggest a protective effect of this hematologic parameter. Ashor and Al-Awamy have shown that HbF may not do so, but they did not compare HbF in those with and without SNHL.7 In their study, the mean HbF in all patients studied was 13.8% ± 1.2 %. Whereas in our study, the mean HbF in those without SNHL was 21.8% ± 8.3%, in contrast to 16.2% ± 8.2% in those with SNHL. In addition, the number of patients in their study was relatively small (42 SCA patients) compared to the number of patients in our study (100 SCA patients). A lower MCV in patients with SNHL with statistically significant differences was not unexpected, as co-existence of SCD and alpha-thalassemia trait is very common in this population.22,23 This finding may suggest that the presence of alpha-thalassemia may increase the risk of SNHL, as it did with some of the complications of SCD, namely osteonecrosis of femoral heads.24Complete recovery in 21.1% of our patients with SNHL and marked improvement in 36.8% suggested the reversibility of this complication. This finding was observed by others.4,5 It is important to emphasize that such acquired SNHL in SCA requires special attention by clinicians dealing with these patients. Diagnosis of SNHL does not mean that these patients will have permanent sequelae. On the other hand, they need better health care, careful follow-up, good education and regular hearing assessment. A careful plan by both the physician and the otolaryngologist should be put forward to suit each patient on his own. The patient may just need reassurance at one end or provision of a hearing aid, and speech or occupational rehabilitation at the other.ARTICLE REFERENCES:1. Morgenstein K, Manace E. Temporal bone histopathology in sickle cell disease . Laryngoscope. 1969; 79: 2172–80. Google Scholar2. Friedman EM, Luban NLC, Herer GR, Williams I. Sickle cell anemia and hearing . Ann Otol Rhinol Laryngol. 1980; 89: 342–7. Google Scholar3. Odetoyinbo O, Adekile A. Sensorineural hearing loss in children with sickle cell anemia . Ann Otol Rhinol Laryngol. 1987; 96: 258–60. Google Scholar4. Urban GE. Reversible sensorineural hearing loss associated with sickle cell crisis . Laryngoscope. 1973; 83: 633–8. Google Scholar5. Orchik DJ, Dunn JW. Sickle cell anemia and sudden deafness . Arch Otolaryngol. 1977; 103: 369–70. Google Scholar6. Todd G, Sergeant G, Larson M. Sensorineural hearing loss in Jamaicans with sickle cell disease . Acta Otolaryngol. 1973; 76: 268–72. Google Scholar7. Ashor A, Al-Awamy B. Sensorineural hearing loss in sickle cell disease in patients in Saudi Arabia . Trop Geog Med. 1985; 37: 314–8. Google Scholar8. Al-Jam'a AH, Al-Dabbous IA. Laboratory diagnosis of patients with sickle cell disease. In: Management Manual of Sickle Cell Disease, 1st ed. Al-Jam'a AH, Al-Dabbous IA, editors. Dammam: Al-Shati Modern Press, 1992: 264–70. Google Scholar9. Jerger J, Jerger S. Measurement of hearing in adults. In: Otolaryngology. Paparella M. and Shumrick D., editors. Philadelphia: W. B. Saunders Co., 1980: 1225–62. Google Scholar10. American National Standard Institute: American National Standards Specification for Audiometers. American Standards Institute Inc., New York, 1969. Google Scholar11. Brookhouser PE, Worthington DW, Kelly WJ. Unilateral hearing loss in children . Laryngoscope. 1991; 101: 1264–72. Google Scholar12. Ajulo SO, Osiname AI, Myatt HM. Sensorineural hearing loss in sickle cell anemia—a United Kingdom study . J Laryngol Otol. 1993; 107: 790–4. Google Scholar13. Atsina KK, Ankra-Badu G. Sensorineural hearing loss in Ghanaians with sickle cell anemia . Trop Geog Med. 1988; 40: 205–8. Google Scholar14. Koide Y, Hando R, Yoshikawa Y. Distribution of some oxidizing enzymes in the cochlea . Acta Otolarngol. 1964; 58: 344–54. Google Scholar15. Serjeant GR, Norman W, Todd GB. The internal auditory canal and sensorineural hearing loss in homozygous sickle cell disease . J Laryngol Otol. 1975; 89: 453–5. Google Scholar16. Perrine RP, Pembrey ME, John P, et al. Natural history of sickle cell anemia in Saudi Arabs: a study of 270 subjects . Ann Intern Med. 1978; 88: 1–6. Google Scholar17. Al-Awami BH, Niazi GA, El-Mouzan MI, et al. Relationship of HbF and alpha-thalassemia to severity of sickle cell anemia in Eastern Province of Saudi Arabia . Ann Trop Pediatr. 1986; 6: 261–5. Google Scholar18. El-Hazmi MAF. Clinical manifestation and laboratory findings of sickle cell anemia in association with alpha-thalassemia in Saudi Arabia . Acta Haematol. 1985; 74: 155–60. Google Scholar19. El-Mouzan MI, Al-Awamy BH, Al-Tourki MT, et al. Variability of sickle cell disease in Eastern Province of Saudi Arabia . J Pediatr. 1989; 114: 973–6. Google Scholar20. El-Hazmi MAF, Warsy AS. Aspects of sickle cell gene in Saudi Arabia. Interaction with glucose-6-phosphate dehydrogenase deficiency . Hum Genet. 1984; 68: 320–3. Google Scholar21. Padmos MA, Roberts GT, Sackey K, et al. Two different forms of homozygous sickle cell disease occur in Saudi Arabia . Br J Haematol. 1991; 79: 93–8. Google Scholar22. El-Hazmi MAF. Hemoglobin disorders: a pattern for thalassemia and hemoglobinopathies in Arabia . Acta Hemat. 1982; 68: 43–51. Google Scholar23. Pembry ME, Wetherall DJ, Glegg JB, et al. Hemoglobin Bart's in Saudi Arabia . Br J Hematol. 1975; 29: 221–34. Google Scholar24. Milner PF, Kraus AP, Sebes JI, et al. Sickle cell disease as a cause of osteonecrosis of femoral head . N Engl J Med. 1991; 325: 1476–81. Google Scholar Previous article Next article FiguresReferencesRelatedDetailsCited byAl Jabr I (2019) Hearing loss among adults with sickle cell disease in an endemic region: a prospective case-control study, Annals of Saudi Medicine , 36:2, (135-138), Online publication date: 1-Mar-2016. Volume 16, Issue 6November 1996 Metrics History Received10 April 1996Accepted28 May 1996Published online1 November 1996 ACKNOWLEDGMENTSWe are pleased to acknowledge Amena Al-Kaiw for performing the audiologic evaluations, Uday Shankar Padmakaran for performing BERA and Luzviminda M. Rosimo for preparation of the manuscript.InformationCopyright © 1996, Annals of Saudi MedicinePDF download" @default.
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