in 1952 , wildervanck , described a cervico - oculo - acoustic syndrome consisting of klippel  feil deformity , abducens palsy with globe retraction ( duane 's retraction ) , and congenital hearing loss . this report describes a case with hypoplastic frontal sinus along with triad of wildervanck syndrome . the patient is a 9-year - old female child admitted to avbr hospital for deformity of an ear , short and deviated neck since birth . anthropometric parameters were suggestive of short stature ( height - 119 cm ; <3 percentile ) . on examination , her vitals were stable . her right eye was smaller than the left eye with an intermittent involuntary decrease in size . it was suggestive of right eyeball retraction . left abducens nerve palsy with short neck and malformed right ear klippel audiometry revealed profound hearing loss on right side and moderate sensorineural hearing loss on the left side . brainstem evoked response audiometry was also suggestive of sensorineural hearing loss on the left side . computed tomography ( ct ) spine was showing of fusion of the thoracic vertebrae , suggestive of klippel  feil deformity . hence , patient 's relatives refused to opt for surgery ; hence , child was referred to higher center with better expertise management for surgery . wildervanck syndrome comprises of the triad of klippel  feil deformity ( fusion of  1 cervical vertebra ) , duane retraction syndrome , and hearing loss . other spinal deformities ( spina bifida occulta , sprengel deformity , and hemivertebrae , fusion of the ribs , absent ribs , kyphosis , scoliosis , and basilar impression ) may coexist . hearing loss in patients with the wildervanck syndrome may be sensorineural , conductive , or mixed and may be accompanied by malformations of the external ear , external acoustic meatus , ossicles , and bony labyrinth . there is a consensus about the mode of inheritance of wildervanck syndrome , but all agree that genetic factors are involved . further , the gene would be partly sex limited acting on a polygenic background , which is modified by sex , rendering females more susceptible than males to action of the gene . an environmental etiology , due to a vascular disruption sequence during embryonic development has been noted in klippel feil anomalies as in moebius and poland sequences . a combination of defects ( kiippel  feil and moebius ) could induce the more complex phenotype observed in wildervanck syndrome . wildervanck stated that deafness should be sensorineural in type ; cases with conductive or mixed losses have also been reported . only one - third of the patients with wildervanck have been described as having hearing loss ; although , audiometry in our patient revealed a moderate degree of sensorineural deafness . wildervanck syndrome with frontal sinus hypoplasia and cholesteatoma is very rare association , which has not yet been described in literature . an expert team work is required for the surgery as intubation may be difficult because of klippel  feil deformity . the possibility of wildervanck should be kept in mind , while evaluating a case of klippel  feil deformity .
we report a case of wildervanck syndrome exhibiting klippel  feil anomaly , duane 's retraction syndrome and congenital deafness . 
 since the first case was reported in 1952 , there have been more reports describing this triad either complete or incomplete . 
 our case has a complete triad of the syndrome along with frontal sinus hypoplasia . 
 our case is unique as the triad was associated with frontal sinus hypoplasia , which is very rare association .