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Bilateral sensorineural hearing impairment

MedGen UID:
96788
Concept ID:
C0452138
Disease or Syndrome
Synonyms: Bilateral sensorineural deafness; Bilateral sensorineural hearing loss; Deafness, bilateral sensorineural; Deafness, sensorineural, bilateral; Hearing impairment, sensorineural, bilateral; Hearing loss, bilateral sensorineural; Hearing loss, bilateral, sensorineural; Hearing loss, sensorineural, bilateral; Sensorineural deafness, bilateral; Sensorineural hearing loss, bilateral
SNOMED CT: Sensorineural hearing loss of bilateral ears (194424005); Sensorineural hearing loss of both ears (194424005)
 
HPO: HP:0008619

Definition

A bilateral form of sensorineural hearing impairment. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBilateral sensorineural hearing impairment

Conditions with this feature

Juvenile myopathy, encephalopathy, lactic acidosis AND stroke
MedGen UID:
56485
Concept ID:
C0162671
Disease or Syndrome
MELAS (mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes) is a multisystem disorder with protean manifestations. The vast majority of affected individuals develop signs and symptoms of MELAS between ages two and 40 years. Common clinical manifestations include stroke-like episodes, encephalopathy with seizures and/or dementia, muscle weakness and exercise intolerance, normal early psychomotor development, recurrent headaches, recurrent vomiting, hearing impairment, peripheral neuropathy, learning disability, and short stature. During the stroke-like episodes neuroimaging shows increased T2-weighted signal areas that do not correspond to the classic vascular distribution (hence the term "stroke-like"). Lactic acidemia is very common and muscle biopsies typically show ragged red fibers.
DOORS syndrome
MedGen UID:
208648
Concept ID:
C0795934
Disease or Syndrome
TBC1D24-related disorders comprise a continuum of features that were originally described as distinct, recognized phenotypes: DOORS syndrome (deafness, onychodystrophy, osteodystrophy, mental retardation, and seizures). Profound sensorineural hearing loss, onychodystrophy, osteodystrophy, intellectual disability / developmental delay, and seizures. Familial infantile myoclonic epilepsy (FIME). Early-onset myoclonic seizures, focal epilepsy, dysarthria, and mild-to-moderate intellectual disability. Progressive myoclonus epilepsy (PME). Action myoclonus, tonic-clonic seizures, progressive neurologic decline, and ataxia. Early-infantile epileptic encephalopathy 16 (EIEE16). Epileptiform EEG abnormalities which themselves are believed to contribute to progressive disturbance in cerebral function. Autosomal recessive nonsyndromic hearing loss, DFNB86. Profound prelingual deafness. Autosomal dominant nonsyndromic hearing loss, DFNA65. Slowly progressive deafness with onset in the third decade, initially affecting the high frequencies.
Autosomal dominant nonsyndromic hearing loss 11
MedGen UID:
331297
Concept ID:
C1832475
Disease or Syndrome
Autosomal dominant deafness-11 is a nonsyndromic form of progressive neurosensory hearing loss with postlingual onset. Some affected individuals have mild vestibular symptoms (summary by Sun et al., 2011).
Syndromic X-linked intellectual disability Shashi type
MedGen UID:
335348
Concept ID:
C1846145
Disease or Syndrome
The Shashi type of X-linked syndromic intellectual developmental disorder (MRXSSH) is characterized by moderately impaired intellectual development and distinctive craniofacial skeletal structure and dysmorphism (Shashi et al., 2015).
Rod-cone dystrophy, sensorineural deafness, and Fanconi-type renal dysfunction
MedGen UID:
376565
Concept ID:
C1849333
Disease or Syndrome
Rod-cone dystrophy, sensorineural deafness, and Fanconi-type renal dysfunction (RCDFRD) is characterized by onset of hearing impairment and reduced vision within the first 5 years of life. Renal dysfunction results in rickets-like skeletal changes, and death may occur in childhood or young adulthood due to renal failure (Beighton et al., 1993).
Acyl-CoA oxidase deficiency
MedGen UID:
376636
Concept ID:
C1849678
Disease or Syndrome
Peroxisomal acyl-CoA oxidase deficiency is a disorder of peroxisomal fatty acid beta-oxidation. See also D-bifunctional protein deficiency (261515), caused by mutation in the HSD17B4 gene (601860) on chromosome 5q2. The clinical manifestations of these 2 deficiencies are similar to those of disorders of peroxisomal assembly, including Zellweger cerebrohepatorenal syndrome (see 214100) and neonatal adrenoleukodystrophy (see 601539) (Watkins et al., 1995).
Deafness, sensorineural, with peripheral neuropathy and arterial disease
MedGen UID:
343766
Concept ID:
C1852280
Disease or Syndrome
Autosomal recessive nonsyndromic hearing loss 67
MedGen UID:
343997
Concept ID:
C1853223
Disease or Syndrome
Any autosomal recessive nonsyndromic deafness in which the cause of the disease is a mutation in the LHFPL5 gene.
Deafness, autosomal dominant 39, with dentinogenesis imperfecta 1
MedGen UID:
340145
Concept ID:
C1854146
Disease or Syndrome
Temtamy preaxial brachydactyly syndrome
MedGen UID:
381425
Concept ID:
C1854466
Disease or Syndrome
Temtamy preaxial brachydactyly syndrome (TPBS) is an autosomal recessive disorder characterized by bilateral, symmetric preaxial brachydactyly and hyperphalangism of digits, facial dysmorphism, dental anomalies, sensorineural hearing loss, delayed motor and mental development, and growth retardation (summary by Li et al., 2010).
Autosomal dominant nonsyndromic hearing loss 20
MedGen UID:
346852
Concept ID:
C1858172
Disease or Syndrome
Any autosomal dominant nonsyndromic deafness in which the cause of the disease is a mutation in the ACTG1 gene.
Anophthalmia/microphthalmia-esophageal atresia syndrome
MedGen UID:
347232
Concept ID:
C1859773
Disease or Syndrome
The phenotypic spectrum of SOX2 disorder includes anophthalmia and/or microphthalmia, brain malformations, developmental delay / intellectual disability, esophageal atresia, hypogonadotropic hypogonadism (manifest as cryptorchidism and micropenis in males, gonadal dysgenesis infrequently in females, and delayed puberty in both sexes), pituitary hypoplasia, postnatal growth delay, hypotonia, seizures, and spastic or dystonic movements.
Spastic paraplegia-nephritis-deafness syndrome
MedGen UID:
355816
Concept ID:
C1866853
Disease or Syndrome
Spastic paraplegia-nephritis-deafness syndrome is a complex form of hereditary spastic paraplegia characterized by progressive, variable spastic paraplegia associated with bilateral sensorineural deafness, intellectual disability, and progressive nephropathy. There have been no further descriptions in the literature since 1988.
Deafness-infertility syndrome
MedGen UID:
370197
Concept ID:
C1970187
Disease or Syndrome
CATSPER-related male infertility results from abnormalities in sperm and can be either CATSPER-related nonsyndromic male infertility (NSMI) or the deafness-infertility syndrome (DIS) when associated with non-progressive prelingual sensorineural hearing loss. Males with NSMI have infertility while females have no symptoms. Males with DIS have both infertility and hearing loss, while females have only hearing loss. Routine semen analysis typically identifies abnormalities in sperm number, morphology, and motility. Otologic examination and audiologic assessment can identify hearing loss.
Autosomal dominant deafness - onychodystrophy syndrome
MedGen UID:
382676
Concept ID:
C2675730
Disease or Syndrome
The DDOD syndrome is characterized by autosomal dominant inheritance of congenital deafness and onychodystrophy. Conical, hypoplastic teeth is also a feature (Robinson et al., 1962). See also DOOR syndrome (220500), an autosomal recessive disorder, which includes congenital deafness, onychodystrophy, osteodystrophy, and mental retardation.
Autosomal recessive nonsyndromic hearing loss 77
MedGen UID:
412541
Concept ID:
C2746083
Disease or Syndrome
Any autosomal recessive nonsyndromic deafness in which the cause of the disease is a mutation in the LOXHD1 gene.
Chromosome 17q12 deletion syndrome
MedGen UID:
482768
Concept ID:
C3281138
Disease or Syndrome
The 17q12 recurrent deletion syndrome is characterized by variable combinations of the three following findings: structural or functional abnormalities of the kidney and urinary tract, maturity-onset diabetes of the young type 5 (MODY5), and neurodevelopmental or neuropsychiatric disorders (e.g., developmental delay, intellectual disability, autism spectrum disorder, schizophrenia, anxiety, and bipolar disorder). Using a method of data analysis that avoids ascertainment bias, the authors determined that multicystic kidneys and other structural and functional kidney anomalies occur in 85% to 90% of affected individuals, MODY5 in approximately 40%, and some degree of developmental delay or learning disability in approximately 50%. MODY5 is most often diagnosed before age 25 years (range: age 10-50 years).
Hearing loss, X-linked 6
MedGen UID:
813067
Concept ID:
C3806737
Disease or Syndrome
Any X-linked nonsyndromic deafness in which the cause of the disease is a mutation in the COL4A6 gene.
Autosomal recessive nonsyndromic hearing loss 101
MedGen UID:
856148
Concept ID:
C3892049
Disease or Syndrome
Any autosomal recessive nonsyndromic deafness in which the cause of the disease is a mutation in the GRXCR2 gene.
Intellectual disability-microcephaly-strabismus-behavioral abnormalities syndrome
MedGen UID:
897984
Concept ID:
C4225351
Disease or Syndrome
White-Sutton syndrome is a neurodevelopmental disorder characterized by a wide spectrum of cognitive dysfunction, developmental delays (particularly in speech and language acquisition), hypotonia, autism spectrum disorder, and other behavioral problems. Additional features commonly reported include seizures, refractive errors and strabismus, hearing loss, sleep disturbance (particularly sleep apnea), feeding and gastrointestinal problems, mild genital abnormalities in males, and urinary tract involvement in both males and females.
Trichothiodystrophy 6, nonphotosensitive
MedGen UID:
934752
Concept ID:
C4310785
Disease or Syndrome
About half of all people with trichothiodystrophy have a photosensitive form of the disorder, which causes them to be extremely sensitive to ultraviolet (UV) rays from sunlight. They develop a severe sunburn after spending just a few minutes in the sun. However, for reasons that are unclear, they do not develop other sun-related problems such as excessive freckling of the skin or an increased risk of skin cancer. Many people with trichothiodystrophy report that they do not sweat.\n\nIntellectual disability and delayed development are common in people with trichothiodystrophy, although most affected individuals are highly social with an outgoing and engaging personality. Some people with trichothiodystrophy have brain abnormalities that can be seen with imaging tests. A common neurological feature of this disorder is impaired myelin production (dysmyelination). Myelin is a fatty substance that insulates nerve cells and promotes the rapid transmission of nerve impulses.\n\nMothers of children with trichothiodystrophy may experience problems during pregnancy including pregnancy-induced high blood pressure (preeclampsia) and a related condition called HELLP syndrome that can damage the liver. Babies with trichothiodystrophy are at increased risk of premature birth, low birth weight, and slow growth. Most children with trichothiodystrophy have short stature compared to others their age. \n\nTrichothiodystrophy is also associated with recurrent infections, particularly respiratory infections, which can be life-threatening. People with trichothiodystrophy may have abnormal red blood cells, including red blood cells that are smaller than normal. They may also have elevated levels of a type of hemoglobin called A2, which is a protein found in red blood cells. Other features of trichothiodystrophy can include dry, scaly skin (ichthyosis); abnormalities of the fingernails and toenails; clouding of the lens in both eyes from birth (congenital cataracts); poor coordination; and skeletal abnormalities including degeneration of both hips at an early age.\n\nThe signs and symptoms of trichothiodystrophy vary widely. Mild cases may involve only the hair. More severe cases also cause delayed development, significant intellectual disability, and recurrent infections; severely affected individuals may survive only into infancy or early childhood.\n\nIn people with trichothiodystrophy, tests show that the hair is lacking sulfur-containing proteins that normally gives hair its strength. A cross section of a cut hair shows alternating light and dark banding that has been described as a "tiger tail."\n\nTrichothiodystrophy, commonly called TTD, is a rare inherited condition that affects many parts of the body. The hallmark of this condition is hair that is sparse and easily broken. 
NAD(P)HX dehydratase deficiency
MedGen UID:
1681210
Concept ID:
C5193026
Disease or Syndrome
Early-onset progressive encephalopathy with brain edema and/or leukoencephalopathy-2 (PEBEL2) is an autosomal recessive severe neurometabolic disorder characterized by rapidly progressive neurologic deterioration that is usually associated with a febrile illness. Affected infants tend to show normal early development followed by acute psychomotor regression with ataxia, hypotonia, and sometimes seizures, resulting in death in the first years of life. Brain imaging shows multiple abnormalities, including brain edema and signal abnormalities in the cortical and subcortical regions (summary by Van Bergen et al., 2019). For a discussion of genetic heterogeneity of PEBEL, see PEBEL1 (617186).
Hearing loss, autosomal recessive 94
MedGen UID:
1679077
Concept ID:
C5193096
Disease or Syndrome
DFNB94 is characterized by prelingual profound sensorineural hearing loss (Simon et al., 2015).
Noonan syndrome 11
MedGen UID:
1681177
Concept ID:
C5193130
Disease or Syndrome
Noonan syndrome (NS) is characterized by characteristic facies, short stature, congenital heart defect, and developmental delay of variable degree. Other findings can include broad or webbed neck, unusual chest shape with superior pectus carinatum and inferior pectus excavatum, cryptorchidism, varied coagulation defects, lymphatic dysplasias, and ocular abnormalities. Although birth length is usually normal, final adult height approaches the lower limit of normal. Congenital heart disease occurs in 50%-80% of individuals. Pulmonary valve stenosis, often with dysplasia, is the most common heart defect and is found in 20%-50% of individuals. Hypertrophic cardiomyopathy, found in 20%-30% of individuals, may be present at birth or develop in infancy or childhood. Other structural defects include atrial and ventricular septal defects, branch pulmonary artery stenosis, and tetralogy of Fallot. Up to one fourth of affected individuals have mild intellectual disability, and language impairments in general are more common in NS than in the general population.
Renal tubular acidosis, distal, 3, with or without sensorineural hearing loss
MedGen UID:
1732975
Concept ID:
C5399980
Disease or Syndrome
Individuals with hereditary distal renal tubular acidosis (dRTA) typically present in infancy with failure to thrive, although later presentations can occur, especially in individuals with autosomal dominant SLC4A1-dRTA. Initial clinical manifestations can also include emesis, polyuria, polydipsia, constipation, diarrhea, decreased appetite, and episodes of dehydration. Electrolyte manifestations include hyperchloremic non-anion gap metabolic acidosis and hypokalemia. Renal complications of dRTA include nephrocalcinosis, nephrolithiasis, medullary cysts, and impaired renal function. Additional manifestations include bone demineralization (rickets, osteomalacia), growth deficiency, sensorineural hearing loss (in ATP6V0A4-, ATP6V1B1-, and FOXI1-dRTA), and hereditary hemolytic anemia (in some individuals with SLC4A1-dRTA).
Kilquist syndrome
MedGen UID:
1742639
Concept ID:
C5436756
Disease or Syndrome
Kilquist syndrome (KILQS) is an autosomal recessive multisystem disorder characterized by neurologic, gastrointestinal, and secretory dysfunction. Affected individuals present at birth with hypotonia, feeding difficulties, mild dysmorphic features, and sensorineural hearing loss. They show poor overall growth associated with gastrointestinal anomalies such as gastroesophageal reflux or midgut malrotation, as well as profound global developmental delay with inability to sit or speak. Tear, sweat, and saliva production is also impaired, causing dry mouth and recurrent bronchial mucus plugging. Some of the clinical features are reminiscent of cystic fibrosis (CF; 219700) (summary by Stodberg et al., 2020).
Delpire-McNeill syndrome
MedGen UID:
1725056
Concept ID:
C5436771
Disease or Syndrome
Delpire-McNeill syndrome (DELMNES) is a neurodevelopmental disorder with highly variable manifestations. Patients present in infancy with global developmental delay, including motor, speech, and impaired intellectual development. The most severely affected patients have hypotonia, inability to hold their head or walk, bilateral sensorineural deafness, and absent language, whereas others have delayed walking and mild to moderate intellectual disability, often with speech delay and autistic features. More variable features may include spasticity or minor involvement of other organ systems, such as hip dislocation or ventricular septal defect (summary by McNeill et al., 2020).
Immunodeficiency 77
MedGen UID:
1788976
Concept ID:
C5543173
Disease or Syndrome
Immunodeficiency-77 (IMD77) is an immunologic disorder characterized by recurrent and persistent polymicrobial infections with multiple unusual organisms. Skin and pulmonary infections are the most common, consistent with increased susceptibility to epithelial cell infections. The age at onset is highly variable: some patients have recurrent infections from childhood, whereas others present in late adulthood. The limited number of reported patients are all female, suggesting incomplete penetrance or a possible sex-influenced trait. Patient cells, mainly macrophages, show impaired killing of intracellular bacteria and organisms, including nontubercular mycobacteria, although there is also impaired killing of other organisms, such as Pseudomonas, Candida, and Aspergillus. Treatment with gamma-IFN (IFNG; 147570) may be a therapeutic option (summary by McCormack et al., 2017 and Merselis et al., 2020).
Neurologic, endocrine, and pancreatic disease, multisystem, infantile-onset 2
MedGen UID:
1778117
Concept ID:
C5543623
Disease or Syndrome
Infantile-onset multisystem neurologic, endocrine, and pancreatic disease-2 (IMNEPD2) is an autosomal recessive multisystemic disorder characterized by cholestatic hepatitis, poor feeding associated with poor overall growth, and hypoglycemia apparent from infancy. Most, but not all, patients have variable global developmental delay. Additional common features include sensorineural deafness, retinal abnormalities with visual defects, and hypotonia. Some patients have endocrine abnormalities, including hyperinsulinemic hypoglycemia, pancreatic dysfunction, hypothyroidism, and primary amenorrhea. Additional features may include hypertriglyceridemia, anemia, proteinuria, increased lactate, and recurrent infections. Brain imaging often shows dysmyelination, thin corpus callosum, cerebral atrophy, and white matter abnormalities. Although the clinical manifestations and severity of the disorder are highly variable, death in early childhood may occur (summary by Williams et al., 2019 and Zeiad et al., 2021). For a discussion of genetic heterogeneity of IMNEPD, see IMNEPD1 (616263).
Spinocerebellar ataxia, autosomal recessive 31
MedGen UID:
1786855
Concept ID:
C5543627
Disease or Syndrome
Autosomal recessive spinocerebellar ataxia-31 (SCAR31) is a complex neurodevelopmental disorder characterized by global developmental delay with hypotonia and variably impaired intellectual and language development. Affected individuals have an ataxic gait, tremor, and dysarthria; more severely affected patients also have spasticity with inability to walk. Most have optic atrophy. Brain imaging shows cerebellar hypoplasia, enlarged ventricles, and atrophy of the posterior corpus callosum. Additional features may include retinitis pigmentosa, sensorineural deafness, dysmorphic facial features, and possibly endocrine dysfunction (summary by Collier et al., 2021).
Developmental delay, impaired speech, and behavioral abnormalities
MedGen UID:
1794167
Concept ID:
C5561957
Disease or Syndrome
Developmental delay, impaired speech, and behavioral abnormalities (DDISBA) is characterized by global developmental delay apparent from early childhood. Intellectual disability can range from mild to severe. Additional variable features may include dysmorphic facial features, seizures, hypotonia, motor abnormalities such as Tourette syndrome or dystonia, and hearing loss (summary by Cousin et al., 2021).
Hearing loss, autosomal dominant 83
MedGen UID:
1812664
Concept ID:
C5676951
Disease or Syndrome
Autosomal dominant deafness-83 (DFNA83) is characterized by the onset of progressive sensorineural hearing loss at an average age of 24 years. A notable finding is a normal distortion product otoacoustic emissions (DPOAE) test, implicating dysfunction of spiral ganglia neurons rather than outer hair cells as a disease mechanism (Cui et al., 2020).
Neurodegeneration, childhood-onset, with multisystem involvement due to mitochondrial dysfunction
MedGen UID:
1824013
Concept ID:
C5774240
Disease or Syndrome
Childhood-onset neurodegeneration with multisystem involvement due to mitochondrial dysfunction (CONDMIM) is an autosomal recessive syndromic disorder characterized primarily by neurologic deficits. Patients show global developmental delay and variably impaired intellectual development with speech delay apparent from infancy. Affected individuals have hypotonia, poor feeding, poor overall growth, and respiratory distress early in life. Other features include visual impairment due to optic atrophy, sensorineural hearing loss, and neuromuscular abnormalities. The severity is highly variable. The disorder is progressive; about half of patients show developmental regression with loss of previous skills. Features suggestive of a mitochondrial disorder include cataracts, cardiomyopathy, diabetes mellitus, combined oxidative phosphorylation deficiency, and increased lactate. Some patients develop seizures, some have dysmorphic facial features, and some have nonspecific abnormalities on brain imaging. Death in childhood may occur (Kaiyrzhanov et al., 2022).

Professional guidelines

PubMed

Rowe SJ
Br J Audiol 1991 Aug;25(4):259-74. doi: 10.3109/03005369109076597. PMID: 1756319

Recent clinical studies

Etiology

Zivkovic Marinkov EM, Rancic NK, Milisavljevic DR, Stankovic MD, Milosevic VD, Malobabic MM, Popovic IN, Ignjatovic AM, Bojanovic MR, Stojanovic JD
Medicina (Kaunas) 2022 Feb 3;58(2) doi: 10.3390/medicina58020233. PMID: 35208557Free PMC Article
Iwanicka-Pronicka K, Trubicka J, Szymanska E, Ciara E, Rokicki D, Pollak A, Pronicki M
Int J Pediatr Otorhinolaryngol 2021 Dec;151:110970. Epub 2021 Nov 10 doi: 10.1016/j.ijporl.2021.110970. PMID: 34775139
Hall JW, Buss E, Grose JH, Roush PA
Ear Hear 2012 May-Jun;33(3):340-8. doi: 10.1097/AUD.0b013e31823fa4c3. PMID: 22237164Free PMC Article
Dietz A, Löppönen T, Valtonen H, Hyvärinen A, Löppönen H
Int J Pediatr Otorhinolaryngol 2009 Oct;73(10):1353-7. Epub 2009 Jul 18 doi: 10.1016/j.ijporl.2009.06.009. PMID: 19616857
Vartiainen E, Kemppinen P, Karjalainen S
Int J Pediatr Otorhinolaryngol 1997 Aug 20;41(2):175-85. doi: 10.1016/s0165-5876(97)00080-3. PMID: 9306174

Diagnosis

Iwanicka-Pronicka K, Trubicka J, Szymanska E, Ciara E, Rokicki D, Pollak A, Pronicki M
Int J Pediatr Otorhinolaryngol 2021 Dec;151:110970. Epub 2021 Nov 10 doi: 10.1016/j.ijporl.2021.110970. PMID: 34775139
Masindova I, Varga L, Stanik J, Valentinova L, Profant M, Klimes I, Gasperikova D
Endocr Regul 2012 Jul;46(3):167-86. doi: 10.4149/endo_2012_03_167. PMID: 22808909
Hall JW, Buss E, Grose JH, Roush PA
Ear Hear 2012 May-Jun;33(3):340-8. doi: 10.1097/AUD.0b013e31823fa4c3. PMID: 22237164Free PMC Article
Dietz A, Löppönen T, Valtonen H, Hyvärinen A, Löppönen H
Int J Pediatr Otorhinolaryngol 2009 Oct;73(10):1353-7. Epub 2009 Jul 18 doi: 10.1016/j.ijporl.2009.06.009. PMID: 19616857
Riga M, Psarommatis I, Lyra Ch, Douniadakis D, Tsakanikos M, Neou P, Apostolopoulos N
Int J Pediatr Otorhinolaryngol 2005 Apr;69(4):449-55. Epub 2005 Jan 4 doi: 10.1016/j.ijporl.2004.11.007. PMID: 15763280

Therapy

Gantz BJ, Perkins R, Murray M, Levy SC, Puria S
Otol Neurotol 2017 Mar;38(3):352-359. doi: 10.1097/MAO.0000000000001300. PMID: 28005723Free PMC Article
Best V, Mason CR, Kidd G Jr, Iyer N, Brungart DS
J Acoust Soc Am 2015 Feb;137(2):EL213-9. doi: 10.1121/1.4907737. PMID: 25698053Free PMC Article
Shen YC, Wang CY, Chen YC, Lee YF
Cornea 2007 Apr;26(3):365-7. doi: 10.1097/ICO.0b013e31802c9e49. PMID: 17413967
Hochermann M, Reimer A
J Laryngol Otol 1987 Oct;101(10):1079-82. doi: 10.1017/s0022215100103263. PMID: 3316458

Prognosis

Lee YH, Tsai CY, Lu YS, Lin PH, Chiang YT, Yang TH, Hsu JS, Hsu CJ, Chen PL, Liu TC, Wu CC
Genes (Basel) 2023 Apr 7;14(4) doi: 10.3390/genes14040880. PMID: 37107638Free PMC Article
Shetty HN, Puttabasappa M
Braz J Otorhinolaryngol 2020 Sep-Oct;86(5):558-567. Epub 2019 Apr 23 doi: 10.1016/j.bjorl.2019.02.010. PMID: 31122881Free PMC Article
Fitzpatrick EM, Olds J, Gaboury I, McCrae R, Schramm D, Durieux-Smith A
Cochlear Implants Int 2012 Feb;13(1):5-15. doi: 10.1179/146701011X12950038111611. PMID: 22340747
Dietz A, Löppönen T, Valtonen H, Hyvärinen A, Löppönen H
Int J Pediatr Otorhinolaryngol 2009 Oct;73(10):1353-7. Epub 2009 Jul 18 doi: 10.1016/j.ijporl.2009.06.009. PMID: 19616857
Vartiainen E, Kemppinen P, Karjalainen S
Int J Pediatr Otorhinolaryngol 1997 Aug 20;41(2):175-85. doi: 10.1016/s0165-5876(97)00080-3. PMID: 9306174

Clinical prediction guides

Shetty HN, Puttabasappa M
Braz J Otorhinolaryngol 2020 Sep-Oct;86(5):558-567. Epub 2019 Apr 23 doi: 10.1016/j.bjorl.2019.02.010. PMID: 31122881Free PMC Article
Fitzpatrick EM, Olds J, Gaboury I, McCrae R, Schramm D, Durieux-Smith A
Cochlear Implants Int 2012 Feb;13(1):5-15. doi: 10.1179/146701011X12950038111611. PMID: 22340747
Kiese-Himmel C
J Laryngol Otol 2008 May;122(5):458-65. Epub 2007 Aug 30 doi: 10.1017/S0022215107000321. PMID: 17727736
Mesolella M, Tranchino G, Nardone M, Motta S, Galli V
Int J Pediatr Otorhinolaryngol 2004 Aug;68(8):995-1005. doi: 10.1016/j.ijporl.2004.02.015. PMID: 15236885
Vartiainen E, Kemppinen P, Karjalainen S
Int J Pediatr Otorhinolaryngol 1997 Aug 20;41(2):175-85. doi: 10.1016/s0165-5876(97)00080-3. PMID: 9306174

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