About Goldenhar's Syndrome

Causes of Goldenhar's Syndrome

    Goldenhar's Syndrome has been blamed on at least three culprits.  The main one is exposure to hazardous chemicals, particularly in the workplace.  Some instances are blamed on viruses during pregnancy.  Still, others appear (RARELY) to be hereditary.

Cause of MY case of Goldenhar's Syndrome

    My case of Goldenhar's Syndrome appears to be caused by my mother's exposure to chemicals in the workplace.  She worked at a chemical plant at the time of my conception (1966).  She does not wish to disclose the name of the plant that she worked at.  Also, she can't remember what chemicals were dealt with at that time.

Prevalence of Goldenhar's Syndrome in
the United States

    Goldenhar's Syndrome is a rare disorder.  In 1967, the year that I was born, there were only about 200 cases in the U.S.  However, after the Gulf War in 1991, this increased due to the chemicals and such that these veterans were exposed to.

Likelihood of Malformed Offspring

    Thank God, this condition is not passed on easily.  The likelihood of an affected parent passing this on to the offspring is about 1 in 200.

Symptoms of Goldenhar's Syndrome

    Symptoms can vary widely.  However, two common features that doctors look for when diagnosing this disorder is facial asymmetry (the two sides of the face not being equally developed) and one or more small ears or ear canals.  In some cases, there may be breathing difficulties, sleep apnea, mental retardation, and other physical abnormalities.  Also, such people are also much more likely to develop allergies, particularly unusual ones.  Here is a list of allergies that I have:

            Eye lubricants (probably all of them)                           Aspirin
            Right-Guard antiperspirant                                          Penicillin
            Zest bar soap                                                             Ancef and/or Demerol
            Black Flag insecticide (even if I'm not using it)             Sulfa drugs
            Roach Motels                                                             Paxil
            Chocolate (only in large amounts)                                Possibly Scotch Guard (even if I'm not
            Surgi-Lube                                                                         using it)

Symptoms That I Had

    The initial symptoms that I had included severe facial deformities, which included double cleft lip, cleft palate, missing eyelids in the left eye, flattened nose, misaligned jaws, and a couple abnormalities of the right ear.  The right ear is slightly out of alignment and had a VERY SMALL canal.  I also have impairments of my speech, hearing, and vision.

    As for my speech, I had extensive speech therapy and did get to where the majority of people can understand me.  It is mainly those with moderate or severe nerve deafness that have the greatest difficulty with me.  HOWEVER, I can kick some butt with my singing, THANK GOD!  I was also plagued with stuttering through much of my life.  When I was a kid, it was intermittent and short-term.  However in 1978, it became chronic, but involved the letter 'B'.  In 1982, it got MUCH worse and involved letters such as CH, D, F, K, P, Q, W, T, and Y--in ADDITION to 'B'.  In early 1984, I had professional speech therapy from a therapist that was qualified to deal with stuttering.  The odd part is that in 1996 when I went to West Virginia University (WVU), most of my stuttering was cured practically overnight.  I think that the reason for this was because when I started going to WVU, I started to live much more independently and was "let out of the cage."

    My hearing was affected by a couple of things--Goldenhar's Syndrome and also a slew of infections in my left ear (otiitis media).  Right now, I can hear about 40-45 decibels on most frequencies.  However, I have a substantial impairment on very low frequencies (below 100 Hz).  As a result, I have an awful time hearing thunder in a closed up house, unless it is practically on top of me.  In my right ear, it takes around 55-65 decibels for me to hear anything.  I wear a Telex in-the-ear (ITE) hearing aid in my left ear, which normally does wonders for me.

    As for my vision, my left eye is basically blind, although I can see objects and color to a certain degree.  My right eye is usually around 20/70, sometimes 20/60.  However, this is a problem with RESOLUTION, not focusing.  Even when fitted with ideal glasses, I am hard pressed to get much better than 20/60.  However, my color vision and night vision are pretty good.  My color vision is so good that a 256-color display looks kind of yucky to me.  I have to have at least 16-bit color (65,536 possible colors) on my computer.  As for reading books, computer screens, or instruments, I just have to get closer than most people.

    Due to my vision and other problems, I have been unable to drive a car safely.  HOWEVER, I do fine in small vehicles or in certain situations, such as driving a tractor to mow grass.  My biggest handicap when driving a car is reading road signs.  While I was fitted with a couple pairs of bioptics (glasses that have a miniature telescope in one or both lenses) and can drive with these, my sleep apnea and possibly my allergy medicine made me a hazard on the road.  Oddly enough, I am able to drive a large (250 cc) gas-powerd two-wheeled scooter, which is much like a motorcycle.  However, I seem to have problems with speeds beyond about 45 MPH or so.

    I also have a STUBBORN case of sleep apnea.  It has defied many treatments, such as CPAP machines, BiPAP machines, nighttime oxygen, and more.  My nighttime oxygen saturation has been known to go down as low as 59%, and this should not be below 90%, and DEFINITELY not below 85%.  I was also known to quit breathing 70 times in ONE HOUR!  However, a tracheostomy with a Montgomery cannula put a swift end to this, but not without some backlashes by "Mr. Sleep Apnea'.

    Needless to say, I had some psychological problems due to this mess.  I have had problems with stuttering and later with depression.

Surgical History

    It is believed that I had about 35-40 surgeries so far in my life.  Most of the surgeries from 1967 to 1980 were done at Riverside Methodist Hospital in Columbus, Ohio.  In 1981 to 1983, I went to the Ohio Valley Medical Center, which is in Wheeling, West Virginia.  This move was prompted by a school speech therapist and for convenience.  However, after a failed jaw surgery, they backed off a ways.  In 1985 to the present, I have been going to Pittsburgh, PA to get things done.  There, I was in Presbyterian University Hospital, Eye & Ear Hospital, and Montefiore Hospital.  These are all connected together in one large complex, which also ties in Children's Hospital, Faulk Clinic, and the Kaufmann Building.

     Also, here is what I CAN ACCOUNT FOR MYSELF when it comes to surgeries:

1972 or 1973 -- Had left eye opened -- partial recollection of this surgery

1973 -- Having my left eye grafted over with skin due to very rapid drying

1973 -- Had drainage tube implanted into left ear (under general anesthesia)

1974 -- Possible repair of pharyngeal flap (at least some sort of mouth surgery)

1978 -- Had left side of lips sowed closed, for opening extended too far to the left

1979 -- Part 1 of removal of excessive lip mass and adding it to the upper lip--required
             a lip tissue in MIDDLE of mouth for about THREE WEEKS!

1979 -- Part 2 of this surgery

1980 -- First nose surgery

1980 -- Smoothing out of lower lip

1981 -- Tumor removal from right eye

1981 -- Opening of left eye

1982 -- Had first eyelid repair done on left eye

1982 -- Jaw surgery #1 -- 7 hours -- A FAILURE!  I was reluctant to have it done.
             So much so that I had thoughts of cutting off my mother's circulation in at least
             one of her arms in order to impair her ability to take me to the hospital for this
             surgery.  I even had thoughts of placing a bunch of cicada shells in her 1977
             Grand Prix to scare her away from driving me to the hospital!

1982 -- Jaw surgery #1's SECOND INFECTION!  This required a 2-3 inch tube to
             implanted into my chin.

1982 -- Undoing of much of jaw surgery #1!  Bone graft was DISSOLVING!

1983 -- Had second eyelid repair done on left eye

1987 -- Had graft taken from right eyelid to better match the color for the left upper

1987 -- Jaw surgery #2 -- 9 hours AND spent a night in HELL (ICU recovery). I darn
             near DIED due to suffocation due to problems caused by an ENDOTRACHEAL
             TUBE!  I was light-headed AND the alarm went off on the pulse oximeter (which
             also measures oxygen saturation)!

1987 -- First infection of jaw surgery #2.  Right side of my face was opened up to drain the

1987 -- Had to surgically drain out pus from the above infection making a bad comeback.

1989 -- Jaw surgery #3 -- came close to losing three good teeth afterwards!

1989 -- Had to get a new canal for my right ear (taken from right leg), due to an anaerobic
             infection, which stemmed from the infection from jaw surgery #2!

1989 -- Had periodontal surgery done

1990 -- Repair of pharyngeal flap, in addition to attempt to repair a hole or two in the roof
             of my mouth (hole(s) opened back up)!

1992 -- Second nose surgery -- GOT INFECTED!

1992 -- Had segment taken from left lower eyelid

1999 -- Had tongue advancement surgery done to help with sleep apnea--just reduce

2000 -- Had a tracheostomy with a Montgomery cannula.  This is a last resort treatment
             for severe sleep apnea

2001 -- Had left eye sewed closed due to it drying out severely AND being allergic to
             every brand of eye lubricant, including the preservative-free variety

2001 -- Second attempt at sewing left eye closed, for it partially opened back up after
             the first attempt

Note that does NOT account for the many surgeries that I had prior to 1972!

    Click HERE to see a spreadsheet consisting of all my surgeries since 1972 and even a few before
that!  You'll also see a 3-D pie chart showing what percentage of my surgeries occurred on which
days of the week!  That's what you call an O.R. special!

    Below is a list of surgeries that I may face in the future:

Another attempt to patch a hole in the roof of my mouth in order to prevent food from
             getting up into my nose.  While this type of surgery is minor, it has a high failure

To make a long story short, I can't seem to keep my hind end out of the operating rooms!  Needless to say, I have been an "O.R. special!"

School History

    In 1972, I was first sent to Crippled Children's School in Elm Grove, West Virginia.  However, a teacher by the name of Sue Langmyer transferred from there to Limestone Elementary, which is in Limestone, West Virginia.  She felt that I had more academic potential than other children at Crippled Children's School and sought to have me transferred to Limestone also.  This permission was granted by the Marshall County Board of Education.

    From 1973 to 1980, I prospered at Limestone Elementary School and performed about average to slightly above average among other kids.  My only academic deficiency that I had was in reading comprehension.  My biggest problem that I had in grade school was a significant number of enemies.  However, I caused much of this problem due to squealing on kids needlessly.  This was a lesson that I learned the hard way.  Below is a picture of this school.

    In 1980 to 1983, I went to Sherrard Junior High, which is in Sherrard, West Virginia.  I was granted permission to go to this school because I went all those years through Limestone Elementary.  My performance was about average to slightly above average.  As for the problem with reading comprehension, this was alleviated by a remedial reading class that I had to take in the seventh grade.  When I went into Sherrard, my reading comprehension was comparable to someone in the tail end of the fourth grade!  When I finished up this class in the seventh grade, I was functioning on a ninth grade level!  In the ninth grade, I majored in Computer Literacy and Industrial Arts and got A's in both.  Luckily, the number of enemies dwindled as I continued to try hard not to be known as a tattle-tale.  Below is a picture of this school.

    From 1983 to 1986, I went to John Marshall High School in Glen Dale, West Virginia.  I did not have to have special permission to go there, for this school is local for Moundsville, Sherrard, Limestone, and Glen Dale students.  Again, I did about average to slightly above average--EXCEPT IN AMERICAN LITERATURE!  My grade point average (GPA) was 2.675 for my high school years.  As for America Literature, I had three nails in my "coffin."  The first one was due to the content not interesting me; Even though I read the stuff, it went "in one eye and out the other!"  The second problem was that we were tested on the little details a lot.  The third problem was that a tough grading scale was used; You had to have a 70% to even get a 'D'.  I majored in computer programming and woodworking.

    From 1986 to 1989, I didn't go to college or work due to the many problems that I had.  With two or more surgeries a year on average, I would have not been able to sustain much when it comes to steady employment and such.  However, I did do a lot of computer programming and such for enjoyment.

    In 1990, I finally started going to college.  I went to Belmont Technical College to be a medical assistant.  I graduated in 1992 with a whopping GPA of 3.72.  However, even after looking for work in FOUR STATES, I could not get a job even close to what I wanted.  I so badly wanted to be an EKG technician.  Below is a picture of this college.

    In 1993, I went to Belmont Technical College again to be a practical nurse.  However, I was having some problems that I didn't realize that I was having.  Instead of my instructors letting me know my faults, they told West Virginia Vocational Rehabilitation (who was paying my way) about this!  As a result, WV Vocational Rehabilitation quit paying my way!

    From 1994 to 1995, I was running around with hardly any sense of direction.  WV Vocational Rehabilitation did say that I could go for schooling in one of four areas that they said I tested well in.  These included computer programming, nuclear medicine, biomedical technician, and electronics repairs.  Shortly afterwards, I decided to go for biomedical technician.  However, I almost lost $35 on an application fee to Penn State when they lied to us about the outlook in this field being good.  In reality, the outlook in the northern panhandle of WV and western PA was POOR!  After finding this out, I was really losing sense of direction.

    In 1996, I started going to WVU.  In early 1996, I could not decide whether to go into Computer Science or Computer Engineering.  After some interviews with several professors, I found that Computer Engineering was more my speed, due to my inventiveness, creativity, and taste for unusual programming.  My GPA normally ran around 2.5 until my depression and burnout really got out of hand, and then it DROPPED--BIG TIME!  In the Fall of 1999, my semester GPA was 0.93!  When I started going to WVU, I had my own apartment and was able to live independently.  Below is a picture of West Virginia University's downtown campus.  It was shot from Arnold Apartments, Apt. 810.


Recommendations for Others with
Goldenhar's Syndrome

    If you or any of your children (if you have any) have Goldenhar's Syndrome or suspect that you have it, PLEASE find a GOOD plastic surgeon.  Don't fart around with some "local guy" just because he's across the street or even a few miles away from home.  While I live in Moundsville, West Virginia, the best choice for me was Pittsburgh, PA--about 65 miles from home.  Also make SURE that your doctor is from a Christian background, for you don't want some money-hungry or scalpel-hungry doctor ripping you open.  Please be aware that while a local plastic surgeon may have gotten very good results on a friend or neighbor, treating problems due to Goldenhar's Syndrome is MUCH more difficult and requires much more artistic surgical skills, as opposed to someone that has been in an accident or horrible crime.  It is IMPERATIVE that the face does not appear as if the person is retarded.  I have had some instances where people thought I was retarded, simply because of my facial deformities.  This even happened at a local flea market when my mother and I were selling things.  Some slime-ball thought he could rip me off, thinking that I could not even count!

My Current Standing

    As of May 18, 2003, I FINALLY graduated from WVU, but not without a hard struggle due to continuing depression (due to severe and ongoing loneliness) and continued problems with allergies.  I am currently looking for suitable employment.  I fired up my own computer business on January 11, 2005.  I do PC repairs and other computer services.  The name of my business is Knoz'em Computers, LLC, which is based in West Virginia (USA).

Go to My Photo Gallery

Return to Main Menu