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Objectives: Although sickle cell disease is the most common of all hereditary disorders, there are a few publications about the effects of the disease on the functions of the ears, nose and throat. In this review, we present an overview of the clinical manifestations of sickle cell disease in general and highlight the problems specifically presenting in the field of otorhinolaryngology., Methods: We review the pathophysiology of sickle cell disease and its clinical features in general. Then, we review its manifestations in ear, nose and throat diseases., Conclusion: Sickle cell disease is one of the most common hemoglobinopathies. It can cause severe pain crises and dysfunction of virtually every organ system in the body, ultimately causing premature death. There is high prevalence (55%) of obstructive adenotonsillar hypertrophy in children and adolescents with sickle cell disease. A very significant reduction has been observed in the rate of pain crises following tonsillectomy in patients with sickle cell anaemia. Prevalence rate for sensorineural hearing loss in older children and adult patients is reporting a range of 11-41%. Priapism of the turbinates is a cause of nasal obstruction in sickle cell anaemia which needs partial turbinectomy. Extramedullary haematopoiesis should be considered in the differential diagnosis of any paranasal sinus mass presenting in a patient with known chronic anaemia., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

Objectives: In poor countries, hearing aids are too expensive for sensorineural hearing loss (SNHL) children's parents to offer for their children. These children may have middle ear problem, this will aggravate the level of hearing loss which may lead to delay in their ability to speak. This study is to highlight the prevalence of middle ear pathology in SNHL children., Methods: Two hundred children with bilateral sensorineural hearing loss (SNHL) were selected in our study from the outpatient clinic of ENT department of Sohag University Hospital, Egypt. Children were classified into three categories according to their middle ear status. They were normal middle ear, middle ear with unhealthy tympanic membrane or otitis media with intact drum and chronic suppurative otitis media with perforation., Results: Seventy percent of cases were normal, 25% had middle ear problem with intact tympanic membrane and 5% had chronic suppurative otitis media with perforation., Conclusion: Thirty percent of SNHL children have middle ear pathology which aggravate the degree of hearing loss. Regular evaluation of SNHL children to treat those having middle ear pathology medically and/or surgically and this may help those having no ability to have hearing aids to learn language early.

Proboscis Lateralis (PL) is one of the congenital anomalies of the nose which presents as obvious deformity. This is a report of two cases of the rare anomaly PL. One presented with right PL and the second presented with left PL. In this rare anomaly, the nasal cavity on one side is completely normal while on the affected side, the nasal cavity is replaced by a tube of skin and soft tissue attached to the inner canthus of the eye. This anomaly occurs sporadic as an isolated defect or in association with other anomalies. It is usually associated with failure of the paranasal sinuses and the nasolacrimal duct development. Embryologically, this anomaly is a result of imperfect fusion of the lateral nasal wall and maxillary processes (Int. J. Pediatr. Otorhinolaryngol. 23 (1992) 275).