Ear Conditions

Examples include:

• Labyrinthitis (inner ear infection that affects your balance)
• Toxoplasmosis
• Cytomegalovirus
• Rubella
• Regular Ear infections (Otitis media)
• Barotitis media
• Lyme disease
• Meningitis
• Middle ear fungal infections
• Alström syndrome
• Auditory neuropathy spectrum disorder (ANSD)
• Cauliflower Ear
• Vertigo
• Meniere’s Disease
• Osteopetrosis
• Otitis Externa
• Otosclerosis
• Perilymphatic fistula
• Polycythaemia vera
• Perichrondritis
• Progeria disease
• Cholesteatoma
• Head injury causing ear condition (ear ringing/ balance and dizziness)
• History of ear/nasal and/or throat infections
• Baby bottle fed (bottle fed have more infections than breast fed)
• Born prematurely before 8 months
• Bell’s palsy
• Ear deafness
• Generalized arterial calcification (infants)
• Glue ear
• Hereditary sensory and autonomic neuropathy type 1E
• NF2-related schwannomatosis (NF2; previously known as neurofibromatosis 2)
• Refsum Disease (Zellweger spectrum disorders)
• Perforated and/or Ruptured Eardrum
• Exostosis (Osteoma)
• Sarcoidosis
• Paget’s disease (affecting skull)
• Lysosomal Lipid Storage and/or Lysosomal Lipid Metabolism Disorders and/or Mucopolysaccharides Metabolism Disorders (Mucopolysaccharidoses)
• Mitochondrial Myopathy/Encephalomyopathy
• Erythromelalgia
• Krabbe disease (affects eyes,ears, muscles,kidney)
• Long Qt syndrome
• Ocular albinism with late onset sensorineural deafness (blue eyes and late deafness)
• Oculo-auriculo-vertebral spectrum [abnormalities tend to involve the cheekbones, jaw, mouth, ears, eyes, and/or bones of the spinal column (vertebrae)]
• Otopalatodigital syndromes type I and II
• Riboflavin transporter deficiency (Brown-Vialetto-Van Laere syndrome, Fazio-Londe syndrome)
• Rubinstein-Taybi syndrome
• Spondyloepiphyseal dysplasia congenita
• Superior semicircular canal dehiscence
• Susac syndrome
• Trismus
• Wegener’s Granulomatosis
• Wolfram syndrome

Gene related conditions

[please note: Genetic causes of hearing loss (not always present at birth- may develop later)]:

• Autosomal recessive disorder (Both parents who are carriers of  the recessive genes responsible and can cause a 25% chance of their sibling getting this condition)
• If both parents are carriers, a child has a 25 percent chance of inheriting two recessive genes (and getting FMF), a 50 percent chance of getting one dominant and one recessive gene (and becoming a carrier), and a 25 percent chance of getting two dominant genes (and remaining unaffected).

Examples include:

• Acrocephalopolysyndactyly disorders (Apert syndrome, Pfeiffer syndrome, Chotzen syndrome, Carpenter syndrome)
• Alport Syndrome
• Branchio-Oto-Renal syndrome
• X-linked Charcot Marie Tooth
• CHARGE syndrome
  • C – Eye coloboma
  • H – congenital heart defects
  • A – choanal atresia-At birth where nasal passages are blocked by bone or tissue.
  • R – retardation of growth/development
  • G – genital anomalies
  • E – ear anomalies/deafness.
• Cornelia de Lange syndrome
• Coffin-Siris syndrome
• Cogan’s syndrome
• Barakat Syndrome (hypoparathyroidism, eyes, ears, kidney, psoriasis)
• Bartter’s syndrome/Gitelman syndrome (affect kidney, ear, muscles)
• Bjornstad syndrome (ear, hair loss)
• Xeroderma Pigmentosum (De Sanctis-Cacchione syndrome)
• DiGeorge Syndrome
• DOORS syndrome (abnormal nails, hearing loss, seizures)
• Downs syndrome
• East syndrome (affect kidney,ear, movement)
• Fountain syndrome (abnormal swelling of cheeks and lips)
• Goodpasture’s syndrome (affect ear, respiratory, kidney)
• Goldenhar disease/syndrome (which affects eye, ear, and spine development)
• Human HOXA1 syndromes (Athabaskan Native Americans, Middle East)
• Jervell and Lange-Nielsen Syndrome– sensorineural hearing loss, cardiac arrhythmias and fainting.
• Johanson-Blizzard syndrome
• Kabuki syndrome
• KAT6B-related disorders
• Klippel-Feil syndrome
• Marshall syndrome
• Mayer-Rokitansky-Küster-Hauser syndrome
• MN1 C-terminal truncation syndrome
• Mondini syndrome-where the cochlea is incomplete, it is associated with Pendred Syndrome, DiGeorge Syndrome, Klippel Feil Syndrome, Fountain Syndrome, Wildervanck Syndrome, CHARGE syndrome, and certain chromosomal trisomies.
• Mohr-Tranebjaerg Syndrome (DFN-1)-sensorineural hearing loss that begins in childhood (usually after a child has learned how to speak) and gets progressively worse, movement problems (involuntary muscle contractions) and/or difficulty swallowing.
• Muckle-Wells syndrome
• Muenke syndrome
• Myhre syndrome
• Norrie Disease
• Homozygous OSMED
• Pendred Syndrome – hearing loss in both ears along with thyroid problems (goiter)
• Perrault syndrome
• PHACE syndrome
• Primary distal renal tubular acidosis
• Rosenberg-Chutorian syndrome
• SeSAME syndrome- Seizures, sensorineural deafness, ataxia, mental retardation, and electrolyte imbalance- low pottasium)
• Smith-Magenis syndrome
• Sotos syndrome
• Stickler syndrome
• Treacher Collins syndrome
• Trichorhinophalangeal syndrome type II (Langer-Giedion syndrome)
• Turners Syndrome
• Waardenburg syndrome – [hearing loss this syndrome can cause eye problems and abnormalities in the pigment (colour) of the hair and eyes]
• Wildervanck syndrome
  • Abnormal union or fusion of two or more bones of the spinal column (vertebrae) within the neck (Klippel-Feil syndrome)
  • Impairment or absence of certain eye (ocular) movements (Duane syndrome)
  • Hearing impairment
• Williams-Beuren syndrome
• Usher Syndrome – (hearing loss, loss of balance and dizziness and vision loss)