CC BY-NC-ND 4.0 · Int Arch Otorhinolaryngol 2020; 24(04): e457-e461
DOI: 10.1055/s-0039-3402442
Original Research

Precochlear Implant Assessment: Clinical Profile and Family History of Children with Severe Bilateral Prelingual Hearing Loss

Syed Hashim Raza
1   Children Hospital, Pakistan Institute of Medical Sciences, Islamabad, Islamabad, Pakistan
,
Rehmana Waris
1   Children Hospital, Pakistan Institute of Medical Sciences, Islamabad, Islamabad, Pakistan
,
Samina Akhtar
2   Department of Diagnostic Radiology, Pakistan Institute of Medical Sciences, Islamabad, Islamabad, Pakistan
,
3   Pakistan Institute of Medical Sciences & Rawalpindi Medical College, Islamabad, Islamabad, Pakistan
4   Atta ur Rahman School of Applied Biosciences, National University of Sciences and Technology, Islamabad, Islamabad, Pakistan
› Author Affiliations

Abstract

Introduction The prevalence of deafness is high in Pakistan. Knowledge regarding the clinical features of patients with profound hearing loss will not only help identify the cause but will also help in the strategic planning for public health interventions.

Objective The present study was conducted to cover in detail the clinical aspects of children with hearing loss, that is, age at presentation, associated deficits and disorders, possible cause of the disease, associated family history, and role of consanguineous marriage.

Methods The present study was performed from November 2016 to September 2018. All of the patients under 6 years of age with profound bilateral hearing loss who would benefit from cochlear implantation were included in the study. Detailed history was taken. The developmental skills were assessed for all areas, and the patients were scored regarding their motor, manipulative, visual, language, social and self-care skills according to the Schedule of Growing Skills II. Detailed family history was taken from the parents of the affected children. The Statistical Package for the Social Sciences (SPSS) software, version 20.0, was used for the statistical analysis.

Results The mean age of the children to be treated was 3.2 ±  1.25 years. Most patients (51.5%) had a positive family history of disease. Consanguineous marriage was common; the parents of 76.9% of the patients were first-degree relatives. Most patients (90.8%) had associated language impediments. In total, four (Ł3.07%) patients had global developmental delay.

Conclusion Consanguineous marriage pattern plays an important role in diseases running in families. Development in these children is strongly linked to their age at the consultation.



Publication History

Received: 17 August 2019

Accepted: 03 November 2019

Article published online:
07 February 2020

© .

Thieme Revinter Publicações Ltda
Rio de Janeiro, Brazil

 
  • References

  • 1 World Health Organization. WHO global estimates on prevalence of hearing loss. World Health Organization; 2012
  • 2 Ali G. Genetic deafness in Pakistani population. J Pak Med Assoc 2010; 60 (06) 418-419
  • 3 Khan MI, Mukhtar N, Saeed SR, Ramsden RT. The Pakistan (Lahore) cochlear implant programme: issues relating to implantation in a developing country. J Laryngol Otol 2007; 121 (08) 745-750
  • 4 Korver AM, Smith RJ, Van Camp G. et al. Congenital hearing loss. Nat Rev Dis Primers 2017; 3 (03) 16094
  • 5 McMahon CM, Kifley A, Rochtchina E, Newall P, Mitchell P. The contribution of family history to hearing loss in an older population. Ear Hear 2008; 29 (04) 578-584
  • 6 Al Khabori M. Causes of severe to profound deafness in Omani paediatric population. Int J Pediatr Otorhinolaryngol 2004; 68 (10) 1307-1313
  • 7 Leal AF. Screening of Patients for Cochlear Implant Through a Questionnaire Online. Group Profile of Patients Pre-and Peri lingual Not Summoned. Int Arch Otorhinolaryngol 2010; 14 (02) 184-191
  • 8 Barbosa MH, Felix F, Ribeiro MG, Tomita S, Pinheiro C, Baptista MM. Profile of patients assessed for cochlear implants. Rev Bras Otorrinolaringol (Engl Ed) 2014; 80 (04) 305-310
  • 9 Bigler D, Burke K, Laureano N, Alfonso K, Jacobs J, Bush ML. Assessment and Treatment of Behavioral Disorders in Children with Hearing Loss: A Systematic Review. Otolaryngol Head Neck Surg 2019; 160 (01) 36-48
  • 10 Hall WC. What you don't know can hurt you: The risk of language deprivation by impairing sign language development in deaf children. Matern Child Health J 2017; 21 (05) 961-965
  • 11 Shojaei E, Jafari Z, Gholami M. Effect of Early Intervention on Language Development in Hearing-Impaired Children. Iran J Otorhinolaryngol 2016; 28 (84) 13-21
  • 12 Ullah S, Aslamkhan M, Ali A, Idrees M. Causes of deafness in the Punjab region of Pakistan and the role of consanguinity. Public Health 2017; 145: 93-95
  • 13 Krug E, Cieza A, Chadha S. et al. Childhood Hearing Loss: Strategies for prevention and care. Switzerland: World Health Organization; 2016: 6-7
  • 14 Akram B, Bashir R. Special Education and Deaf Children in Pakistan: An Overview. Journal of Elementary Education. 2012; 22 (02) 33-34
  • 15 Schafer E, Utrup A. The Effect of Age of Cochlear Implantation on Speech Intelligibility to Others. JEPRA 2016; 22: 1-11