Cochlear implant in Thalassemia patient – Case report

Abstract

Introduction: Beta-thalassemias are a group of hereditary blood disorders characterized by anomalies in the synthesis of the beta chains of hemoglobin.

Iron overload occurs in thalassemia, with blood transfusion therapy being the major cause. Deferoxamine continues to be the mainstay of therapy to remove excess iron in patients requiring long-term transfusions.

One of the most important complications of deferoxamine therapy is neurosensory toxicity, including sensorineural hearing loss (SNHL).

Labyrinthine hemmorhage (LH) is thought to result from altered capillary hemodynamics or reperfusion injury. It is theorized that LH incites a reparative response that cascades from fibrosis to sclerosis and ultimately ossification of the inner ear structures.

Case presentation: We present a case of 3-year-old thalassemic child with bilateral profound sensorineural hearing loss. Patient was on regular blood transfusions with chelation therapy.

HRCT temporal bone and MRI brain and temporal bone had features of labyrinthitis ossificans (LO).

Child underwent uniateral cochlear implantation and postimplantation speech perception and production outcomes were normal.

Discussion: This case illustrates the unique feature of labyrinthitis ossificans in a thalassemia patient which has not yet reported in the English literature.

Hearing screening of all thalassemia patients and therefore early diagnosis of SNHL prompts early intervention and improved quality of life.

Keywords:

• Cochlear implant
• Thalassemia
• Sensorineural hearing loss
• Labyrinthitis ossificans
• Deferoxamine
• Hearing screening

ORCID

Shitanshu Sharma http://orcid.org/0000-0002-9660-5367

Notes on contributors

Tanmaya Kataria is a junior resident pursuing postgraduation in the Department of Otorhinolaryngology at SMS Medical College & Hospitals, Jaipur, Rajasthan, India.

Mohnish Grover is Associate Professor in the Department of Otorhinolaryngology at SMS Medical College & Hospitals, Jaipur, Rajasthan, India, and is a well-known cochlear implant surgeon in India.

Shitanshu Sharma is a senior resident in the Department of Otorhinolaryngology at SMS Medical College & Hospitals, Jaipur, Rajasthan, India, and has published three papers national as well as international.

Sunil Samdani is Senior Professor in the Department of Otorhinolaryngology at SMS Medical College & Hospitals, Jaipur, Rajasthan, India, and is a famous otologist and specialist in airway foreign bodies.

Man Prakash Sharma is Senior Professor and Head of Department in the Department of Otorhinolaryngology at SMS Medical College & Hospitals, Jaipur, Rajasthan, India, and is a famous surgeon for his prosperous work and hospitality towards patients.