background: major beta thalassemia is the most common inherited anemia with high prevalence in iran and hearing loss is one of its side effects. the present study aimed to determine the hearing status of patients with major thalassemia and its relationship with serum ferritin level, period of blood transfusion and dessfroxamin administration. methods: this descriptive-analytic study was perform...

Introduction: The present study aimed to investigate the audiological profiles of elementary school-age children in Rasht, Iran, and estimate the prevalence of hearing impair­ments in this population.   Materials and Methods: In this cross-sectional descriptive-analytical study, the hearing threshold was screened using pure tone audiomet...

An implantable bone conduction hearing device can be of significant benefit to carefully selected patients with noncorrectable conductive hearing losses. However, for some patients the device has significant limitations. This paper presents several practical issues that need to be considered before a decision is made regarding implant surgery. It is recommended that, whenever possible, air cond...

Unilateral Conductive or Mixed Hearing Loss Unilateral implantable bone conduction (bone-anchored) hearing aid may be considered medically necessary as an alternative to an air-conduction hearing aid in patients 5 years of age and older with a conductive or mixed hearing loss when ONE of the following medical criteria is met:  Congenital or surgically induced malformations (eg, atresia) of the...

Osteogenesis imperfecta (OI) is an inherited bone and connective tissue disorder associated with the lifelong occurrence of frequent fractures following even mild trauma. Hearing loss is frequently reported in patients with OI. Objective: to examine the ratio of hearing loss in children with OI, and the relationship between audiological findings and CT images of temporal bone in children with O...

OBJECTIVE A lack of attention has been given to hearing health in primary care in developing countries. A strategy involving low-cost screening tools may fill the current gap in hearing health care provided to children. Therefore, it is necessary to establish and adopt lower-cost procedures that are accessible to underserved areas that lack other physical or human resources that would enable th...

BACKGROUND PHACE syndrome describes a spectrum of anomalies associated with large facial infantile hemangiomas and characterized by posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities. With improved recognition and imaging practices of infants with PHACE syndrome, additional associations have been identified. To our...

BACKGROUND The inherited bone marrow failure syndromes (IBMFSs) are diverse disorders with syndrome-specific features; their otologic and audiologic manifestations have not been well described. Our objective was to characterize these in patients with Fanconi anemia (FA), dyskeratosis congenita (DC), Diamond-Blackfan anemia (DBA), and Shwachman-Diamond syndrome (SDS), and to determine the associ...

SUMMARY A subset of patients presents with unilateral conductive hearing loss, a normal pinna or grade I microtia, and mild external auditory canal stenosis. The physical findings of microtia and a small external canal are commonly absent or subtle in this group of patients, who are being commonly referred for imaging to evaluate isolated conductive hearing loss. We present a case series of pat...